Hyperinsulinism malady

Wikipedia: Hyperinsulinism refers to an above normal level of insulin in the blood of a person or animal. Normal...⁴⁴ more...

MalaCards: Hyperinsulinism, also known as hyperinsulinemia, is related to persistent hyperinsulinemic hypoglycemia of infancy and hyperinsulinism-hyperammonemia syndrome. An important gene associated with Hyperinsulinism is GLUD1 (glutamate dehydrogenase 1), and among its related pathways are Translation Insulin regulation of translation and Selected targets of GCR-alpha. The compounds pegvisomant and diazoxide have been mentioned in the context of this disorder. Affiliated tissues include liver, ovary and t cells, and related mouse phenotypes are endocrine/exocrine gland and hematopoietic system.

Aliases & Descriptions:

hyperinsulinism 6 7 8 32 43 hyperinsulinemia 6 32

hyperinsulinism (disorder) 6 hyperinsulinism nos 6

Diseases related to hyperinsulinism by text searches and GeneDecks gene sharing:

(show top 50)    (show all 509)

id	Related Disease	Score	Top Affiliating Genes
1	persistent hyperinsulinemic hypoglycemia of infancy	34.7	GCK, KCNJ11, GLUD1, ABCC8, HADH
2	hyperinsulinism-hyperammonemia syndrome	34.5	KCNJ5, INS, GLUD1, GLUD1P5, GLUL
3	insulin resistance	32.4	LEPR, UCP2, IRS1, INS
4	polycystic ovary syndrome	31.0	LEP, IRS1, IRS2, INS, INSR, IGFBP1
5	acute insulin response	30.9	GCG, KCNJ11, KCNJ5, UCP2, INS, GLUD1
6	glucose intolerance	30.5	GCG, APOB, GHRL, SLC2A2
7	3-hydroxyacyl-coa dehydrogenase deficiency	29.9	GCK, INS, HSD17B10, GLUD1, HADH
8	hyperinsulinemic hypoglycemia	28.5	INS, IGF2, SST, ABCC8
9	21-hydroxylase deficiency	28.3	BRD2, GNRH1, SULT2A1, POMC
10	acanthosis nigricans	27.9	LEP, INS, INSR, IGF1, GH1, SHBG
11	ischemic heart disease	27.9	RETN, SERPINE1, LPL, LIPC, KCNJ5, APOB
12	acid-labile subunit	27.9	IGFBP1, IGF2, IGFBP3, IGF1, GH1, GHR
13	adrenal hyperplasia	27.6	GCG, LEP, INS, IGFBP3, IGF1, GNRH1
14	hypothyroidism	27.5	RETN, LEP, APOB, IGFBP3, GHRL, SHBG
15	short stature	27.4	LEP, INS, IGFBP1, IGF2, IGFBP3, IGF1
16	autonomic dysfunction	27.1	SERPINE1, INS, INSR, PHOX2B, NPY
17	alcoholic liver cirrhosis	26.8	INS, INSR, GPT, IGFBP1, IGFBP3, IGF1
18	glycogen storage disease	26.8	GCG, LPL, INS, IGF2, IGF1, HNF1A
19	carotid intimal medial thickness	26.3	SERPINE1, LPL, INS, PPARG
20	insulinoma	26.3	GCK, GCG, KCNJ11, KCNJ5, LEPR, UCP2
21	thalassemia	26.3	SELE, VCAM1, LEP, INS, GPT, IGFBP3
22	hyperandrogenism	26.3	RETN, BRD2, LEP, IRS1, IRS2, INS
23	alcoholism	26.2	GPT, GLUD1, GHRL, POMC, NPY
24	glomerulopathy	26.1	SELE, LEP, LEPR, INS, INSR, APOB
25	gestational diabetes	25.9	RETN, GCK, GCG, KCNJ11, LEP, LEPR
26	hyperuricemia	25.9	GCK, SERPINE1, LPL, LIPC, LEP, INS
27	hypertriglyceridemia	25.7	GCKR, SELE, SERPINE1, LPL, VCAM1, LIPC
28	maturity-onset diabetes of the young	25.6	GCKR, GCK, GCG, KCNJ11, IRS1, INS
29	liver cirrhosis	25.4	RETN, GCG, LEPR, IRS1, INSR, GPT
30	familial combined hyperlipidemia	25.3	LPL, LIPC, LEPR, INS, APOB, PPARA
31	hypoglycemia	25.1	GCKR, GCK, GCG, BRD2, LPL, KCNJ11
32	essential hypertension	25.0	RETN, GCK, SELE, SERPINE1, LPL, VCAM1
33	lipodystrophy	24.1	RETN, SERPINE1, LPL, LIPC, LEP, INS
34	hyperlipidemia	24.0	RETN, GCK, SERPINE1, LPL, VCAM1, LIPC
35	uremia	23.5	RETN, SERPINE1, LPL, VCAM1, LIPC, LEP
36	neuropathy	23.4	RETN, GCG, SELE, SERPINE1, LEP, UCP2
37	aldosteronism	23.3	RETN, SERPINE1, KCNJ5, IRS1, IRS2, INS
38	anemia	23.0	SELE, LPL, VCAM1, LEP, INS, GPT
39	nonalcoholic steatohepatitis	23.0	RETN, SERPINE1, LPL, LEP, LEPR, FOXO1
40	obesity, association with	22.9	RETN, SERPINE1, LEP, LEPR, UCP2, INS
41	endometrial carcinoma	22.8	SERPINE1, VCAM1, LEPR, FOXO1, IRS1, INSR
42	hypercholesterolemia	22.7	RETN, GCG, SELE, SERPINE1, LPL, VCAM1
43	osteoarthritis	22.5	RETN, SERPINE1, BRD2, VCAM1, LEP, INS
44	acromegaly	21.9	RETN, GCG, SERPINE1, BRD2, LPL, LEP
45	type 2 diabetes mellitus	21.2	RETN, GCKR, GCK, GCG, SELE, SERPINE1
46	anorexia nervosa	21.0	RETN, GCG, SERPINE1, LPL, LEP, LEPR
47	immunodeficiency	21.0	RETN, GCG, SELE, SERPINE1, VCAM1, LEP
48	coronary heart disease	20.7	RETN, GCKR, GCG, SELE, SERPINE1, LPL
49	atherosclerosis	20.2	RETN, GCKR, GCK, GCG, SELE, SERPINE1
50	hypertension	19.9	RETN, GCK, SELE, SERPINE1, LPL, VCAM1

Approved drugs:

Drug clinical trials:

MalaCards organs/tissues related to hyperinsulinism:

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MGI Mouse Phenotypes related to hyperinsulinism:

²⁵ (show all 22)

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	endocrine/exocrine gland phenotype	MP:0005379	10.5	BRD2, KCNJ11, GNRH1, GLUD1, GHR, ABCC8
2	hematopoietic system phenotype	MP:0005397	10.4	HK1, IGF2, GPR83
3	renal/urinary system phenotype	MP:0005367	9.4	H19, HADH, HK1, DDC, GHR, HNF1A
4	integument phenotype	MP:0010771	9.2	POMC, HK1, EGR1, GNRH1, IRS2, LEPR
5	respiratory system phenotype	MP:0005388	9.0	PHOX2B, INSR, UCP2, LEPR, LPL
6	digestive/alimentary phenotype	MP:0005381	8.7	HNF1A, GHRL, SST, EDN1, NPY, GNRH1
7	immune system phenotype	MP:0005387	8.6	POMC, HK1, PPARA, GHR, GNRH1, GPR83
8	skeleton phenotype	MP:0005390	8.1	AKT1, HNF1A, PPY, PPARG, EGR1, H19
9	no phenotypic analysis	MP:0003012	8.1	GHRL, SST, PPARG, POMC, H19, GGPS1
10	reproductive system phenotype	MP:0005389	8.1	HK1, GH1, HNF1A, AKT1, GNRH1, MAPK1
11	liver/biliary system phenotype	MP:0005370	7.8	RETN, GCK, BRD2, LPL, IRS1, INS
12	normal phenotype	MP:0002873	7.4	HNF4A, GHRL, SST, PPARG, PHOX2B, NPY
13	tumorigenesis	MP:0002006	7.4	H19, POMC, EGR1, PPARG, AKT1, GNRH1
14	nervous system phenotype	MP:0003631	7.2	AKT1, HNF4A, GHR, DDC, PHOX2B, EDN1
15	muscle phenotype	MP:0005369	6.2	IGFBP3, IGF1, AKT1, HNF1A, PPARG, PPARA
16	behavior/neurological phenotype	MP:0005386	5.4	GHRL, GHR, HNF1A, AKT1, GLUL, IGFBP3
17	adipose tissue phenotype	MP:0005375	5.3	INSR, MC3R, MC4R, FASN, IGFBP3, IGF1
18	cellular phenotype	MP:0005384	4.9	IGF1, GNRH1, AKT1, HNF1A, HNF4A, DDC
19	mortality/aging	MP:0010768	4.4	DDC, GHR, HNF4A, AKT1, GLUL, SREBF1
20	growth/size phenotype	MP:0005378	4.0	MAPK3, H19, HADH, SLC2A2, MAPK1, GNRH1
21	cardiovascular system phenotype	MP:0005385	3.8	MAPK1, IGF2, IGF1, AKT1, GHR, DDC
22	homeostasis/metabolism phenotype	MP:0005376	0.9	RETN, INSR, APOB, MC3R, MC4R, FASN

Articles related to hyperinsulinism:

(show top 50)    (show all 174)

id	Title	Authors	Year	Affiliating Genes
1	The contribution of rapid KATP channel gene mutation analysis to the clinical management of children with congenital hyperinsulinism . (21378087)	Banerjee I.... Clayton P.E.	2011	ABCC8, KCNJ11
2	Spectrum of glutamate dehydrogenase mutations in Japa nese patients with congenital hyperinsulinism and hyperammonemia syndrome. (22106762)	Aso K.... Shintaku H.	2011	GLUD1
3	An early response transcription factor, Egr-1, enhanc es insulin resistance in type 2 diabetes with chronic hyperinsulinism. (21321112)	Shen N.... Li C.J.	2011	MAPK3, MAPK1, EGR1
4	Mutation analysis of the GLUD1 gene in patients with glutamate dehydrogenase congenital hyperinsulinism]. (20931523)	Sang Y.M.... Gu Y.	2010	GLUD1
5	On the reversibility of glutamate dehydrogenase and t he source of hyperammonemia in the hyperinsulinism/hyperammonemia syndrome. (19895831)	Treberg J.R.... Brosnan J.T.	2010	GLUD1
6	Changes in cytokine levels during acute hyperinsuline mia in offspring of type 2 diabetic subjects. (20031127)	Ruotsalainen E.... Laakso M.	2010	SELE, VCAM1
7	Congenital hyperinsulinism due to a compound heterozy gous ABCC8 mutation with spontaneous resolution at eight weeks. (20215776)	Kumaran A.... Hussain K.	2010	ABCC8, KCNJ11
8	Neurological aspects of hyperinsulinism-hyperammonaemia syndrome. (19046187)	Bahi-Buisson N.... Heinrichs C.	2008	GLUD1
9	Mutations in UCP2 in congenital hyperinsulinism reveal a role for regulation of insulin secretion. (19065272)	Gonzalez-Barroso M.M.... Ricquier D.	2008	UCP2, INS, KCNJ5
10	Destabilization of ATP-sensitive potassium channel activity by novel KCNJ11 mutations identified in congenital hyperinsulinism. (18250167)	Lin Y.W.... Shyng S.L.	2008	ABCC8, KCNJ11, INS
11	Congenital central hypoventilation syndrome with hyperinsulinism in a preterm infant. (18340402)	Hennewig U.... Hoehn T.	2008	PHOX2B
12	Insights in congenital hyperinsulinism. (17986831)	Hussain K.	2007	GCK, GLUD1, ABCC8
13	Insulin, C-peptide and proinsulin for the biochemical diagnosis of hypoglycaemia related to endogenous hyperinsulinism. (17609405)	Vezzosi D.... Caron P.	2007	INS
14	Rapid genetic analysis in congenital hyperinsulinism. (17114887)	Christesen H.B.... Jacobsen B.B.	2007	KCNJ11
15	Congenital hyperinsulinism associated ABCC8 mutations that cause defective trafficking of ATP-sensitive K+ channels: identification and rescue. (17575084)	Yan F.F.... Shyng S.L.	2007	ABCC8, KCNJ11
16	Genotype-phenotype associations in patients with severe hyperinsulinism of infancy. (17378627)	Greer R.M.... Cotterill A.M.	2007	ABCC8, KCNJ11
17	Mutations in the genes encoding the pancreatic beta-cell KATP channel subunits Kir6.2 (KCNJ11) and SUR1 (ABCC8) in diabetes mellitus and hyperinsulinism. (16416420)	Gloyn A.L.... Ellard S.	2006	ABCC8, KCNJ11
18	A novel KCNJ11 mutation associated with congenital hyperinsulinism reduces the intrinsic open probability of beta-cell ATP-sensitive potassium channels. (16332676)	Lin Y.-W.... Shyng S.-L.	2006	ABCC8, KCNJ11
19	The Knudson's two-hit model and timing of somatic mutation may account for the phenotypic diversity of focal congenital hyperinsulinism. (16882742)	Giurgea I.... de Lonlay P.	2006	KCNJ11
20	Genotype-phenotype correlations in children with congenital hyperinsulinism due to recessive mutations of the adenosine triphosphate-sensitive potassium channel genes. (15562009)	Henwood M.J.... Stanley C.A.	2005	ABCC8, KCNJ11, INS
21	Massive insulin secretion in response to anaerobic exercise in exercise-induced hyperinsulinism. (16308838)	Meissner T.... Mayatepek E.	2005	INS
22	Hyperinsulinism of infancy associated with a novel splice site mutation in the SCHAD gene. (15870679)	Hussain K.... Eaton S.	2005	HSD17B10, HADH
23	Genetics and pathophysiology of hyperinsulinism in infancy. (14981344)	Cosgrove K.E.... Dunne M.J.	2004	GCK, HADH
24	Infantile hyperinsulinism associated with enteropathy, deafness and renal tubulopathy: clinical manifestations of a syndrome caused by a contiguous gene deletion located on chromosome 11p. (15645695)	Hussain K.... Aynsley-Green A.	2004	USH1C
25	The genetics of neonatal hyperinsulinism. (12566718)	Fournet J.C.... Junien C.	2003	GLUD1
26	Acute insulin response tests for the differential diagnosis of congenital hyperinsulinism. (12364426)	Huopio H.... Otonkoski T.	2002	ABCC8, KCNJ11, INS
27	Chronic hyperinsulinism induced down-regulation of insulin post-receptor signaling transduction in Hep G2 cells. (12674767)	Yuan L.... Hamann A.	2002	INSR, IRS1
28	Protein-sensitive and fasting hypoglycemia in childre n with the hyperinsulinism/hyperammonemia syndrome. (11241047)	Hsu B.Y.... Stanley C.A.	2001	GLUD1
29	Unbalanced expression of 11p15 imprinted genes in focal forms of congenital hyperinsulinism: association with a reduction to homozygosity of a mutation in ABCC8 or KCNJ11. (11395395)	Fournet J.C.... Junien C.	2001	ABCC8, KCNJ11, H19
30	Hyperinsulinism of infancy: the regulated release of insulin by KATP channel-independent pathways. (11272144)	Straub S.G.... Dunne M.J.	2001	ABCC8, SST, INS
31	Hyperinsulinism/hyperammonemia syndrome in children with regulatory mutations in the inhibitory guanosine triphosphate-binding domain of glutamate dehydrogenase. (11297618)	MacMullen C.... Van Vliet G.	2001	GLUD1, GLUD1P5
32	Acute insulin responses to leucine in children with the hyperinsulinism/hyperammonemia syndrome. (11502802)	Kelly A.... Stanley C.A.	2001	GLUD1, INS
33	Contribution of total and intact proinsulins to hyperinsulinism in subjects with obesity, impaired glucose tolerance or type 2 diabetes. (11011219)	BlicklAc J.F.... AndrA"s E.	2000	INS
34	Dominantly inherited hyperinsulinism caused by a mutation in the sulfonylurea receptor type 1. (11018078)	Huopio H.... Otonkoski T.	2000	ABCC8, KCNJ11
35	Sensitization to insulin in adolescent girls to normalize hirsutism, hyperandrogenism, oligomenorrhea, dyslipidemia, and hyperinsulinism after precocious pubarche. (11061495)	IbA!A+ez L.... de Zegher F.	2000	INS
36	Loss of imprinted genes and paternal SUR1 mutations lead to focal form of congenital hyperinsulinism. (10895035)	Fournet J.C.... Junien C.	2000	ABCC8
37	Hyperinsulinism of infancy: towards an understanding of unregulated insulin release. European Network for Research into Hyperinsulinism in Infancy. (10685980)	Shepherd R.M.... Dunne M.J.	2000	INS
38	Genetics of neonatal hyperinsulinism. (10685979)	Glaser B.... Junien C.	2000	GLUD1
39	Hyperinsulinism and its interaction with hyperandrogenism in polycystic ovary syndrome. (10804539)	Sozen I.... Arici A.	2000	INSR, INS
40	Intracellular hyperinsulinism: a metabolic characteristic of obesity with and without Type 2 diabetes: intracellular insulin in obesity and Type 2 diabetes. (10624789)	Benzi L.... Navalesi R.	1999	INSR
41	Congenital hyperinsulinism: molecular basis of a heterogeneous disease. (10338089)	Meissner T.... Mayatepek E.	1999	GLUD1, ABCC8, KCNJ11
42	Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene. (9571255)	Stanley C.A.... Poncz M.	1998	GLUD1, GLUD1P5
43	Familial hyperinsulinism caused by an activating glucokinase mutation. (9435328)	Glaser B.... Herold K.C.	1998	GCK
44	Hyperinsulinism in infants and children. (9130925)	Stanley C.A.	1997	ABCC8
45	The relationship between fatty liver and hyperinsulin emia in obese Japanese children. (9138179)	Kawasaki T.... Uchiyama M.	1997	GPT
46	Hyperinsulinism in children: diagnostic value of pancreatic venous sampling correlated with clinical, pathological and surgical outcome in 25 cases. (8545179)	Dubois J.... Saudubray J.M.	1995	SST
47	Familial hyperinsulinism maps to chromosome 11p14-15.1, 30 cM centromeric to the insulin gene. (7920639)	Glaser B.... Stanley C.A.	1994	ABCC8
48	Arterial hypertension, hyperinsulinism and insulin resistance (1438100)	Ribstein J.... Mimran A.	1992	INS
49	Hyperinsulinism and the coronary syndrome (1494868)	Hrnciar J.	1992	EDN1
50	Total pancreatectomy in a case of nesidioblastosis due to persisting hyperinsulinism following subtotal pancreatectomy. (1904602)	Dohrmann P.... Splieth J.	1991	SST

Genes related to hyperinsulinism according to GeneCards:

(show top 50)    (show all 72)

id	Symbol	Description	Score	GeneCards Section Context
1	GLUD1	glutamate dehydrogenase 1	11.1	Publications, Summaries
2	KCNJ11	potassium inwardly-rectifying channel, subfamily J, member 11	11.0	Publications
3	ABCC8	ATP-binding cassette, sub-family C (CFTR/MRP), member 8	11.0	Publications
4	HADH	hydroxyacyl-CoA dehydrogenase	11.0	Publications
5	KCNJ5	potassium inwardly-rectifying channel, subfamily J, member 5	11.0	Publications
6	GCK	glucokinase (hexokinase 4)	11.0	Publications
7	INS	insulin	11.0	Publications
8	GLUD1P5	glutamate dehydrogenase 1 pseudogene 5	11.0	Publications
9	HSD17B10	hydroxysteroid (17-beta) dehydrogenase 10	11.0	Publications
10	GLUL	glutamate-ammonia ligase	11.0	Publications
11	IGFBP1	insulin-like growth factor binding protein 1	11.0	Publications
12	SHBG	sex hormone-binding globulin	11.0	Publications
13	SST	somatostatin	11.0	Publications
14	INSR	insulin receptor	11.0	Publications
15	LSL	Leptin, serum levels of	11.0	Publications
16	GNRH1	gonadotropin-releasing hormone 1 (luteinizing-releasing hormone)	11.0
17	GHRL	ghrelin/obestatin prepropeptide	11.0	Publications
18	IGF1	insulin-like growth factor 1 (somatomedin C)	11.0	Publications
19	LEP	leptin	11.0	Publications
20	IGFBP3	insulin-like growth factor binding protein 3	11.0	Publications
21	GH1	growth hormone 1	11.0
22	GHR	growth hormone receptor	11.0
23	IGF2	insulin-like growth factor 2 (somatomedin A)	11.0
24	SIRT4	sirtuin 4	11.0	Publications
25	SAR1A	SAR1 homolog A (S. cerevisiae)	11.0	Publications
26	GGPS1	geranylgeranyl diphosphate synthase 1	11.0	Publications
27	PPY	pancreatic polypeptide	11.0
28	PHOX2B	paired-like homeobox 2b	11.0	Publications
29	USH1C	Usher syndrome 1C (autosomal recessive, severe)	11.0	Publications
30	GPR83	G protein-coupled receptor 83	11.0
31	H19	H19, imprinted maternally expressed transcript (non-protein coding)	11.0	Publications
32	MC3R	melanocortin 3 receptor	11.0
33	GCKR	glucokinase (hexokinase 4) regulator	11.0	Publications
34	GIP	gastric inhibitory polypeptide	11.0
35	PYGL	phosphorylase, glycogen, liver	11.0
36	SULT2A1	sulfotransferase family, cytosolic, 2A, dehydroepiandrosterone (DHEA)-preferring, member 1	11.0
37	UCP2	uncoupling protein 2 (mitochondrial, proton carrier)	11.0	Publications
38	PYGB	phosphorylase, glycogen; brain	11.0
39	BRD2	bromodomain containing 2	11.0
40	CPS1	carbamoyl-phosphate synthase 1, mitochondrial	11.0	Publications
41	MC4R	melanocortin 4 receptor	11.0
42	RNF130	ring finger protein 130	11.0
43	DDC	dopa decarboxylase (aromatic L-amino acid decarboxylase)	11.0	Publications
44	HNF1A	HNF1 homeobox A	11.0	Publications
45	SLC2A2	solute carrier family 2 (facilitated glucose transporter), member 2	11.0
46	SREBF1	sterol regulatory element binding transcription factor 1	11.0
47	FOXO1	forkhead box O1	11.0
48	IRS2	insulin receptor substrate 2	11.0
49	EGR1	early growth response 1	11.0	Publications
50	KCNJ12	potassium inwardly-rectifying channel, subfamily J, member 12	11.0

Pathways related to hyperinsulinism according to GeneDecks:

(show top 50)    (show all 94)

id	Pathway	Score	Top Affiliating Genes
1	Translation Insulin regulation of translation10	10.7	IRS1
2	Selected targets of GCR-alpha10	10.5	IGFBP1, GHR, GLUL, INS, INSR, LEP
3	Development_Leptin signaling via JAK/STAT and MAPK cascades41	10.4	EGR1, LEPR
4	Aldosterone-regulated sodium reabsorption20	10.4	IRS2, MAPK3, INS
5	Maturity onset diabetes of the young20	10.3	SLC2A2, GCK, HNF1A, HNF4A
6	Glucose transport38	10.3	GCKR, GCK, HK1, SLC2A2
7	Glycogen metabolism41	10.2	GCK, PYGB, PYGL, HK1
8	Glycogen metabolism10	10.2	HK1, PYGB, GCK, PYGL
9	Adipocytokines & Insulin Signaling37	10.1	IRS1, INS, INSR, IRS2, RETN, LEP
10	Selected targets of HNF110	10.1	SLC2A2, UCP2, HNF1A, INS, APOB, IGFBP1
11	MODY (Maturity-Onset Diabetes of Young)36	10.1	SLC2A2, INSR, HNF4A, INS, GCK
12	Type II diabetes mellitus20	10.0	GCK, KCNJ11, IRS1, IRS2, INS, MAPK3
13	Galactose metabolism41	9.7	HK1, PYGL, PYGB, GCK
14	Development Leptin signaling via PI3K-dependent pathway10	9.7	KCNJ11, LEPR, ABCC8, IRS2, IRS1, AKT1
15	Development_Leptin signaling via PI3K-dependent pathway41	9.6	IRS1, KCNJ11, LEP, LEPR, IRS2, ABCC8
16	Anti-diabetic Drug Potassium Channel Inhibitors Pathway, Pharmacodynamics34	9.5	KCNJ11, GCK, SLC2A2, ABCC8, HNF4A, IRS1
17	Growth Hormone Signaling36	9.5	IGF2, GH1, MAPK1, GHR, IRS1, IGF1
18	AMPK Enzyme Complex Pathway36	9.5	IRS2, IRS1, INS, FASN, INSR, AKT1
19	Transcription Receptor-mediated HIF regulation10	9.4	MAPK1, IGFBP3, IRS1, IRS2, INS, INSR
20	Translation _Regulation of EIF2 activity41	9.4	MAPK1, INSR, IRS2, IRS1, AKT1
21	Development_Growth hormone signaling via PI3K/AKT and MAPK cascades41	9.2	IRS1, EGR1, GHR, GH1, AKT1, MAPK1
22	Development Growth hormone signaling via PI3K/AKT and MAPK cascades10	9.2	IRS1, IRS2, MAPK1, AKT1, GH1, GHR
23	Development IGF-RI signaling10	9.1	IGFBP3, IGF2, IGFBP1, MAPK1, MAPK3, IRS1
24	eIF2 Pathway36	9.1	INS, GH1, AKT1, GNRH1, IGF1, IGF2
25	Regulation of lipid metabolism Insulin signaling-generic cascades10	9.1	AKT1, IRS2, MAPK1, MAPK3, INSR, INS
26	Regulation of lipid metabolism_Insulin signaling-generic cascades41	9.1	MAPK1, MAPK3, INSR, IRS2, IRS1, INS
27	Translation_Insulin regulation of translation41	9.1	MAPK1, MAPK3, INSR, INS, IRS2, IRS1
28	IL-7 Signaling Pathways37	9.1	AKT1, MAPK1, MAPK3, IRS2, IRS1
29	Translation Regulation activity of EIF210	9.1	IRS1, IRS2, INS, INSR, MAPK3, MAPK1
30	Activation of PKA through GPCR36	9.1	PYGL, PYGB, GH1, GNRH1, IGF2, IGF1
31	Transcription_Receptor-mediated HIF regulation41	9.0	IRS1, IRS2, INS, INSR, MAPK3, MAPK1
32	IL-2 Signaling Pathways37	9.0	MAPK3, IRS2, IRS1, MAPK1, AKT1, FOXO1
33	Common Cytokine Receptor Gamma-Chain Family Signaling Pathways37	9.0	MAPK3, FOXO1, IRS1, IRS2, AKT1, MAPK1
34	Prostate cancer20	9.0	MAPK1, IGF1, AKT1, FOXO1, INS, MAPK3
35	Insulin Receptor Pathway36	9.0	AKT1, MAPK1, INSR, IRS1, IRS2, MAPK3
36	Development_IGF-1 receptor signaling41	9.0	IGFBP1, IGFBP3, MAPK1, IRS2, IRS1, IGF1
37	mTOR Pathway36	9.0	INS, IRS1, IGF2, IGF1, GH1, AKT1
38	p70S6K Signaling36	8.9	IRS1, IGF1, IGF2, GH1, AKT1, MAPK1
39	Glioblastoma Multiforme36	8.9	FOXO1, MAPK1, IGF2, AKT1, MAPK3
40	IGF1R Signaling36	8.9	IRS2, IGF1, AKT1, IRS1, IGF2, MAPK1
41	Development Thrombopoietin-regulated cell processes10	8.8	MAPK1, FOXO1, IRS2, MAPK3, AKT1
42	Development_Thrombopoietin-regulated cell processes41	8.7	MAPK1, MAPK3, IRS2, FOXO1, AKT1
43	Development Leptin signaling via JAK/STAT and MAPK cascades10	8.7	EDN1, LEP, LEPR, MAPK3, MAPK1, EGR1
44	PPAR Pathway36	8.6	AKT1, MAPK1, IGF2, GH1, IGF1, PPARG
45	ILK Signaling36	8.6	GH1, AKT1, GNRH1, IGF1, IGF2, MAPK1
46	Transcription PPAR Pathway10	8.6	MAPK1, MAPK3, PPARA, PPARG, AKT1
47	Glucose / Energy Metabolism3	8.6	INSR, SREBF1, AKT1, GCG, HK1, FASN
48	Adipocytokine signaling pathway20	8.5	NPY, IRS2, IRS1, POMC, PPARA, AKT1
49	Transcription_PPAR Pathway41	8.3	PPARA, PPARG, AKT1, MAPK3, INSR, IRS1
50	Insulin signaling pathway20	8.2	IRS2, AKT1, PYGB, PYGL, SREBF1, HK1

Compounds related to hyperinsulinism according to GeneDecks:

(show top 50)    (show all 352)

id	Compound	Score	Top Affiliating Genes
1	pegvisomant32 9 9	12.4	SST, GHRL, GHR, GH1, GLUL, IGF1
2	diazoxide32 42 9 9	13.3	GH1, GLUL, GLUD1, IGFBP3, INS, KCNJ5
3	acipimox32	10.0	LSL, SST, GHRL, GH1, IGF1, APOB
4	sulfonylurea32	10.0	IRS1, KCNJ5, KCNJ11, GCG, GCK, SLC2A2
5	glimepiride32 34 9 9	12.6	ABCC8, PPARG, INSR, INS, KCNJ5, KCNJ11
6	carbohydrates32	9.6	NPY, GHR, FASN, UCP2, VCAM1, SELE
7	dehydroepiandrosterone sulfate32	9.5	POMC, LSL, SHBG, SULT2A1, GHRL, GHR
8	androstenedione32 18	10.2	LEP, POMC, LSL, SHBG, GHR, GH1
9	intralipid32	9.1	LEP, LIPC, LPL, INS, APOB, LSL
10	progesterone32 42 9 18 9	13.1	SLC2A2, EGR1, SULT2A1, GHR, GH1, GNRH1
11	isoproterenol32 9 9	11.0	INSR, UCP2, GCG, MAPK3, MAPK1, SST
12	estrone32 9 18 9	12.0	LSL, SHBG, SULT2A1, GH1, GNRH1, IGF1
13	rapamycin32 42	9.9	IGF2, FASN, INS, IRS2, IRS1, FOXO1
14	estradiol32 9 18 9	11.8	SERPINE1, POMC, SHBG, SULT2A1, IGFBP1, FASN
15	corticosterone32 18	9.8	GCG, LEP, LEPR, NPY, POMC, LSL
16	acth32	8.8	LEP, SST, SULT2A1, GHRL, GHR, GNRH1
17	triiodothyronine32	8.7	LEP, LIPC, LEPR, UCP2, POMC, LSL
18	aspartate32	8.6	AKT1, GLUL, GLUD1, GNRH1, FASN, KCNJ11
19	dihydrotestosterone32 9 18 9	11.6	SHBG, GHR, GH1, HSD17B10, GNRH1, IGF1
20	palmitate32	8.4	PPARA, PPARG, GHR, HNF4A, FASN, MC4R
21	dopamine32 9 18 9	11.3	EGR1, PHOX2B, SST, DDC, SULT2A1, GHRL
22	orlistat32 42 9 9	11.1	GCG, LPL, LIPC, NPY, LSL, PPARG
23	losartan32 34 9 9	11.0	EGR1, EDN1, PPARG, AKT1, IGF1, IGFBP3
24	gnrh32	7.9	AKT1, IGF1, IGFBP3, IGFBP1, MC3R, INS
25	fatty acid32	7.9	HADH, HK1, ABCC8, LSL, PPARA, GHRL
26	epinephrine32 9 18 9	10.6	NPY, POMC, EGR1, LSL, SHBG, DDC
27	ly29400232	7.6	SREBF1, AKT1, MAPK1, FASN, IRS2, IRS1
28	steroid32	7.5	HNF1A, GLUL, HSD17B10, GNRH1, IGF1, IGF2
29	vitamin d32	7.4	LEPR, LPL, SERPINE1, INSR, GPT, SHBG
30	ibmx32	7.1	SLC2A2, EDN1, GCG, LPL, PPARA, PPARG
31	creatinine32	7.0	EDN1, LSL, SHBG, PPARA, PYGB, GHRL
32	pd 98,05932	6.9	IRS1, LEP, VCAM1, SERPINE1, GCG, IRS2
33	phosphatidylinositol32	6.9	EDN1, LSL, SREBF1, GHR, GIP, HNF4A
34	indomethacin32 9 9	8.8	NPY, POMC, EGR1, EDN1, PPARA, PPARG
35	tamoxifen32 34 9 9	9.8	EGR1, IRS2, IRS1, LEP, VCAM1, LPL
36	thyroxine32 18	7.6	NPY, POMC, LSL, SHBG, PPARG, SST
37	testosterone32 9 18 9	9.6	SREBF1, SST, SULT2A1, GHR, HNF4A, AKT1
38	glutamate32	6.5	GNRH1, IGFBP3, IGF2, IGFBP1, GPT, MAPK1
39	glutamine32	6.2	PPARG, LEPR, KCNJ11, LPL, GCG, GCK
40	norepinephrine32 9 18 9	9.2	GHRL, GCG, RETN, SELE, LPL, GH1
41	metformin32 34 9 9	9.0	POMC, INS, IRS2, IRS1, LEP, KCNJ11
42	adenylate32	5.7	NPY, POMC, EGR1, SHBG, SST, VCAM1
43	rosiglitazone32 9 18 9	8.7	GH1, IGFBP3, IGFBP1, GPT, MAPK1, APOB
44	genistein32 9 18 9	8.1	EGR1, EDN1, SHBG, PPARA, PPARG, SST
45	alanine32	4.4	GLUD1, LEPR, VCAM1, SELE, GCG, GCK
46	vegf32	4.4	RETN, IGFBP1, MAPK1, MAPK3, FASN, APOB
47	serine32	4.2	IGFBP3, VCAM1, SERPINE1, SELE, GCG, GCK
48	cycloheximide32	4.0	POMC, EGR1, FASN, APOB, INSR, IRS2
49	dexamethasone32 42 34 9 9	7.8	POMC, EGR1, LSL, SHBG, PPARA, PPARG
50	estrogen32	2.9	LIPC, VCAM1, LPL, SERPINE1, GCG, RETN

Cellular components related to hyperinsulinism according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	secretory granule	GO:030141	9.5	POMC, GHRL, GIP, INS, GCG, GCK
2	caveola	GO:005901	8.8	MAPK1, MAPK3, INSR, IRS1, SELE
3	extracellular region	GO:005576	6.6	GIP, GH1, GHR, GHRL, SST, PPY
4	extracellular space	GO:005615	6.4	GCG, IGF1, GNRH1, GH1, GHR, GHRL
5	cytosol	GO:005829	6.3	GLUL, AKT1, PYGL, SULT2A1, DDC, PPARG
6	plasma membrane	GO:005886	5.9	APOB, MC3R, MC4R, FASN, GPR83, HSD17B10

Biological processes related to hyperinsulinism according to GeneDecks:

(show all 48)

id	Name	GO ID	Score	Top Affiliating Genes
1	positive regulation of glycolysis	GO:045821	10.7	IGF1, INSR, INS, GCK
2	positive regulation of MAPK cascade	GO:043410	10.5	IGF1, IGFBP3, IGF2, INSR, INS, LEP
3	positive regulation of insulin receptor signaling pathway	GO:046628	10.5	IGF2, INS, IRS1, LEP
4	cellular glucose homeostasis	GO:001678	10.5	GCKR, GCK, FOXO1
5	positive regulation of peptidyl-tyrosine phosphorylation	GO:050731	10.5	GHR, GH1, IGF1, IGF2, INS
6	negative regulation of insulin secretion	GO:046676	10.5	HADH, SIRT4, GHRL, IRS1, KCNJ11
7	positive regulation of insulin secretion	GO:032024	10.5	GCK, IRS2, GLUD1, GIP, GHRL
8	positive regulation of insulin-like growth factor receptor signaling pathway	GO:043568	10.4	IGFBP3, IGF1, GH1
9	positive regulation of mitosis	GO:045840	10.4	EDN1, IGF1, IGF2, INSR, INS
10	adult feeding behavior	GO:008343	10.3	LEP, GHRL, NPY
11	response to amino acid stimulus	GO:043200	10.3	CPS1, EDN1, SST, GIP
12	response to food	GO:032094	10.2	CPS1, GHR, AKT1
13	insulin-like growth factor receptor signaling pathway	GO:048009	10.1	GHR, AKT1, IGF1, IRS1
14	negative regulation of appetite	GO:032099	10.1	GCG, LEP, PPARA
15	hexose transport	GO:008645	10.1	GCKR, GCK, HK1, SLC2A2
16	regulation of insulin secretion	GO:050796	10.1	SLC2A2, ABCC8, GIP, HNF4A, INS, LEP
17	positive regulation of glucose metabolic process	GO:010907	10.1	IRS1, IRS2, AKT1
18	response to glucose stimulus	GO:009749	10.0	SLC2A2, EGR1, GIP, HNF4A, GLUL, INSR
19	positive regulation of nitric oxide biosynthetic process	GO:045429	10.0	EDN1, AKT1, INSR, INS
20	leukocyte tethering or rolling	GO:050901	10.0	SELE, VCAM1, LEP
21	positive regulation of glucose import	GO:046326	10.0	AKT1, IGF1, INSR, INS, IRS2, IRS1
22	positive regulation of tyrosine phosphorylation of Stat5 protein	GO:042523	10.0	GHR, GH1, IGF1
23	carbohydrate metabolic process	GO:005975	9.9	SLC2A2, HK1, ABCC8, PYGL, PYGB, INSR
24	positive regulation of fatty acid beta-oxidation	GO:032000	9.9	PPARA, IRS2, IRS1
25	positive regulation of glycogen biosynthetic process	GO:045725	9.9	AKT1, IGF1, IGF2, INSR, INS, IRS2
26	glucose metabolic process	GO:006006	9.9	PYGL, PYGB, GHRL, AKT1, IGF2, INS
27	response to insulin stimulus	GO:032868	9.8	HADH, EGR1, PPARA, MC4R, IRS1, LEP
28	endocrine pancreas development	GO:031018	9.8	SLC2A2, GIP, HNF4A, AKT1, INS, FOXO1
29	positive regulation of cell migration	GO:030335	9.8	IRS1, IRS2, INS, INSR, EDN1
30	phosphatidylinositol-mediated signaling	GO:048015	9.8	AKT1, IGF1, IRS2, IRS1, FOXO1
31	positive regulation of cell proliferation	GO:008284	9.7	EDN1, IGF1, IGF2, INSR, INS, IRS2
32	glucose transport	GO:015758	9.7	SLC2A2, HK1, EDN1, GH1, AKT1, INS
33	cholesterol metabolic process	GO:008203	9.7	SREBF1, APOB, LEPR, LEP, LIPC
34	cellular nitrogen compound metabolic process	GO:034641	9.7	CPS1, POMC, DDC, GLUL, GLUD1, HSD17B10
35	lipid homeostasis	GO:055088	9.6	PPARG, HNF4A, IRS2
36	energy reserve metabolic process	GO:006112	9.6	FASN, GIP, ABCC8, SLC2A2, MC4R, INS
37	activation of MAPK activity	GO:000187	9.6	GHRL, GHR, MAPK1, MAPK3, INSR
38	JAK-STAT cascade involved in growth hormone signaling pathway	GO:060397	9.5	GHR, GH1, MAPK1, MAPK3, IRS2, IRS1
39	cellular response to insulin stimulus	GO:032869	9.5	PPARG, GHR, AKT1, INSR, IRS2, IRS1
40	triglyceride homeostasis	GO:070328	9.4	GIP, HNF4A, LIPC, LPL, GCKR
41	triglyceride catabolic process	GO:019433	9.3	CPS1, APOB, LIPC, LPL
42	synaptic transmission	GO:007268	9.2	NPY, ABCC8, SST, GLUL, MAPK1, KCNJ5
43	glucose homeostasis	GO:042593	9.0	PPARG, PYGL, HNF4A, HNF1A, AKT1, INSR
44	insulin receptor signaling pathway	GO:008286	8.9	AKT1, IGF2, IGFBP1, MAPK1, MAPK3, INSR
45	response to drug	GO:042493	8.7	HADH, CPS1, EDN1, PPARG, SST, GIP
46	lipid metabolic process	GO:006629	8.4	PPARA, PPARG, SREBF1, HNF4A, HSD17B10, APOB
47	positive regulation of transcription from RNA polymerase II promoter	GO:045944	7.5	POMC, EGR1, PHOX2B, PPARA, PPARG, SREBF1
48	small molecule metabolic process	GO:044281	6.2	GCKR, PYGB, PYGL, SULT2A1, DDC, PPARA

Molecular functions related to hyperinsulinism according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	phosphatidylinositol 3-kinase binding	GO:043548	10.1	IRS1, IRS2, INSR
2	NAD+ binding	GO:070403	10.0	GLUD1, SIRT4, HADH
3	insulin-like growth factor receptor binding	GO:005159	10.0	IGF1, IGF2, INSR, INS, IRS1
4	insulin receptor binding	GO:005158	9.8	SLC2A2, IGF1, IGF2, INS, IRS2, IRS1
5	hormone activity	GO:005179	8.9	RETN, POMC, EDN1, PPY, SST, GH1
6	protein binding	GO:005515	3.2	GH1, HNF4A, HNF1A, AKT1, GLUD1, HSD17B10