Progeria malady

NIH Rare Diseases: Progeria is a rare condition characterized by dramatic, rapid aging beginning in childhood. Affected newborns usually appear normal but within a year, their growth rate slows significantly. Affected children develop a distinctive appearance characterized by baldness, aged-looking skin, a pinched nose, and a small face and jaw relative to head size. They also often have symptoms typically seen in much older people including joint stiffness, hip dislocations and severe, progressive cardiovascular disease. Intelligence is typically normal. The average lifespan is age 13; death is usually due to heart attack or stroke. Progeria is caused by mutations in the LMNA gene, but almost always results from a new mutation rather than being inherited from a parent. Management focuses on the individual signs and symptoms of the condition. Although there is currently no cure, research involving treatment is ongoing and progress is being made.³⁰

MalaCards: Progeria, also known as HGPS, is related to cockayne syndrome and mandibuloacral dysplasia. An important gene associated with Progeria is LMNA (lamin A/C), and among its related pathways are Granzyme Pathway and Apoptosis and survival_Caspase cascade. The compounds 8-hydroxyadenine and 8-hydroxyguanine have been mentioned in the context of this disorder. Affiliated tissues include skin, heart and t cells, and related mouse phenotypes are respiratory system and pigmentation.

Genetics Home Reference: Hutchinson-Gilford progeria syndrome is a genetic condition characterized by the dramatic, rapid appearance of aging beginning in childhood. Affected children typically look normal at birth and in early infancy, but then grow more slowly than other children and do not gain weight at the expected rate (failure to thrive). They develop a characteristic facial appearance including prominent eyes, a thin nose with a beaked tip, thin lips, a small chin, and protruding ears. Hutchinson-Gilford progeria syndrome also causes hair loss (alopecia), aged-looking skin, joint abnormalities, and a loss of fat under the skin (subcutaneous fat). This condition does not disrupt intellectual development or the development of motor skills such as sitting, standing, and walking.¹⁷

Wikipedia: Progeria (also known as \"Hutchinson–Gilford (Progeria) Syndrome\", and \"Progeria syndrome\") is an...⁴⁴ more...

OMIM: 176670

GeneReviews summary for hgps

Aliases & Descriptions:

progeria 6 44 30 17 8 32 43 hgps 6 44 15 30 16 17 hutchinson-gilford progeria syndrome 6 44 15 17 hutchinson gilford progeria syndrome 44 30 16 43 hutchinson gilford syndrome 6 15 30

hutchinson-gilford disease 6 16 hutchinson-gilford syndrome (disorder) 6 progeria syndrome (disorder) 6 hutchinson-gilford progeria 33 progeria (disorder) 6

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

(show top 50)    (show all 162)

id	Related Disease	Score	Top Affiliating Genes
1	cockayne syndrome	29.0	XPA, GTF2H4, ERCC1, ERCC4, ERCC6, TP53
2	mandibuloacral dysplasia	28.7	LMNA, ZMPSTE24, INS
3	werner syndrome	28.4	LMNA, INS, TP53, HELLS, WRN, ADIPOQ
4	xeroderma pigmentosum, group a	27.2	LMNA, XPA, ERCC4, TP53
5	ataxia telangiectasia	27.1	XPA, TP53, HELLS, SOD2, WRN
6	muscular dystrophy	27.0	LMNA, LMNB1, LMNB2, BANF1, INS, SUN1
7	myopathy	26.0	SELE, LMNA, LMNB1, INS, SYNE2, SOD2
8	lipodystrophy	26.0	LMNA, LMNB1, LMNB2, ZMPSTE24, INS, EMD
9	xeroderma pigmentosum	25.9	LMNA, XPA, GTF2H4, ERCC1, ERCC4, ERCC6
10	hyperglycemia	25.6	SELE, INS, ENPP1, RNF130, SOD2, ADIPOQ
11	atrial fibrillation	25.3	SELE, LMNA, INS, TNFRSF11B, EMD
12	atherosclerosis	22.5	SELE, LMNA, BANF1, INS, ENPP1, ERCC6
13	tight skin contracture syndrome, lethal	13.7	LMNA, ZMPSTE24
14	restrictive dermopathy	13.7	LMNA, ZMPSTE24
15	emerinopathy	13.6	LMNA, EMD
16	reynolds syndrome	13.6	LMNB1, LMNB2, ZMPSTE24
17	pelger-huet anomaly	13.6	LMNA, LMNB2
18	photosensitive trichthiodystrophy	13.3	XPA, GTF2H4, ERCC4
19	xeroderma pigmentosum, group f	13.3	ERCC1, ERCC4
20	cerebrooculofacioskeletal syndrome	13.2	ERCC1, ERCC6
21	vulvar cancer	13.1	TP53, HELLS, WRN
22	photosensitive trichothiodystrophy	13.0	XPA, ERCC1, ERCC6, HELLS
23	atypical werner syndrome	13.0	LMNA, INS, WRN
24	arthropathy	12.9	LMNA, ZMPSTE24, TNFRSF11B, ACAN
25	familial partial lipodystrophy	12.9	LMNA, INS, EMD
26	emery-dreifuss muscular dystrophy	12.8	LMNA, LMNB1, LMNB2, BANF1, SUN1, SYNE2
27	congenital heart defect	12.8	LMNA, SOD2, EMD, ADIPOQ
28	breast cancer susceptibility	12.7	ERCC1, TP53, HELLS, SOD2, WRN
29	breast cancer	12.7	ERCC1, TP53, HELLS, SOD2, WRN
30	calcinosis	12.7	LMNA, ZMPSTE24, ENPP1
31	congenital generalized lipodystrophy	12.5	LMNA, ZMPSTE24, INS, ADIPOQ
32	acanthosis nigricans	12.5	LMNA, INS, ADIPOQ
33	prostate carcinoma	12.5	ERCC1, ERCC4, ERCC6, TP53
34	prediabetes syndrome	12.4	INS, ADIPOQ
35	dwarfism	12.3	INS, ERCC4, ERCC6, ACAN
36	glucose intolerance	12.2	LMNA, INS, ENPP1, ADIPOQ, GGT1
37	skin cancer	12.2	XPA, ERCC1, ERCC4, ERCC6, TP53, SOD2
38	arrhythmogenic right ventricular dysplasia/cardiomyopathy multi-gene panels	12.2	LMNA, INS, ANK3, ELN, EMD
39	21-hydroxylase deficiency	12.2	INS, ADIPOQ, GGT1
40	hyperhomocysteinemia	12.2	SELE, INS, ELN, SOD2, GGT1
41	fascioliasis	12.2	SELE, INS, GGT1
42	obesity, association with	12.2	INS, ENPP1, ADIPOQ, GGT1
43	gestational diabetes	12.1	INS, ENPP1, ADIPOQ
44	abdominal aortic aneurysm	12.1	SELE, TNFRSF11B, ELN, HELLS, ADIPOQ
45	myotonic dystrophy	12.1	LMNA, INS, ERCC1, ADIPOQ, GGT1
46	cataract	12.1	LMNA, INS, ERCC1, ERCC6, SOD2, WRN
47	diabetic angiopathy	12.0	SELE, INS, TNFRSF11B, SOD2
48	diabetes, type 2	12.0	SELE, INS, ADIPOQ
49	viral hepatitis	12.0	INS, TNFRSF11B, SOD2, ADIPOQ, GGT1
50	diffuse large b-cell lymphoma	11.9	SELE, LMNA, ERCC1, ERCC4, TP53, WRN

Clinical features from OMIM: 176670

Approved drugs:

Drug clinical trials:

MalaCards organs/tissues related to progeria:

²²

MGI Mouse Phenotypes related to progeria:

²⁵ (show all 23)

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	respiratory system phenotype	MP:0005388	9.5	PGGT1B, SYNE2, TWIST2, ELN, B4GALT1, LMNB2
2	pigmentation phenotype	MP:0001186	9.2	GGT1, SOD2, HELLS, TP53, XPA, LMNA
3	craniofacial phenotype	MP:0005382	8.5	TP53, TNFRSF11B, ACAN, TWIST2, XPA, ZMPSTE24
4	limbs/digits/tail phenotype	MP:0005371	8.3	ACAN, HELLS, TWIST2, WRN, GGT1, TNFRSF11B
5	liver/biliary system phenotype	MP:0005370	8.2	PGGT1B, TWIST2, ACAN, TP53, ERCC6, ERCC4
6	tumorigenesis	MP:0002006	8.0	ADIPOQ, EEF1E1, WRN, PGGT1B, SOD2, NLRP12
7	digestive/alimentary phenotype	MP:0005381	8.0	TWIST2, ACAN, NLRP12, TP53, INS, B4GALT1
8	integument phenotype	MP:0010771	7.8	WRN, LMNB2, LMNB1, ZMPSTE24, XPA, B4GALT1
9	skeleton phenotype	MP:0005390	7.6	WRN, HELLS, ACAN, SPG7, TNFRSF11B, TP53
10	vision/eye phenotype	MP:0005391	7.4	TWIST2, SOD2, WRN, GGT1, TP53, ERCC6
11	renal/urinary system phenotype	MP:0005367	7.4	ADIPOQ, SOD2, TWIST2, HELLS, TP53, ERCC1
12	endocrine/exocrine gland phenotype	MP:0005379	7.2	GGT1, ADIPOQ, WRN, TWIST2, SUN1, TP53
13	muscle phenotype	MP:0005369	7.0	SUN1, ELN, TWIST2, SYNE2, SOD2, EMD
14	adipose tissue phenotype	MP:0005375	7.0	TP53, HELLS, TWIST2, SOD2, WRN, ADIPOQ
15	cardiovascular system phenotype	MP:0005385	6.4	ELN, TWIST2, SOD2, WRN, EMD, ADIPOQ
16	nervous system phenotype	MP:0003631	6.2	ERCC6, TP53, SPG7, ACAN, SYNE2, SOD2
17	behavior/neurological phenotype	MP:0005386	5.4	SPG7, ACAN, HELLS, TWIST2, SOD2, EMD
18	hematopoietic system phenotype	MP:0005397	5.3	TNFRSF11B, NLRP12, HELLS, TWIST2, SOD2, PGGT1B
19	homeostasis/metabolism phenotype	MP:0005376	5.3	ACAN, ELN, HELLS, TWIST2, TAF10, SOD2
20	immune system phenotype	MP:0005387	4.7	TWIST2, HELLS, ACAN, NLRP12, SOD2, PGGT1B
21	growth/size phenotype	MP:0005378	4.6	TNFRSF11B, SUN1, SPG7, ACAN, HELLS, TWIST2
22	cellular phenotype	MP:0005384	4.3	TAF10, TWIST2, HELLS, NLRP12, SYNE2, SOD2
23	mortality/aging	MP:0010768	3.2	GGT1, ADIPOQ, EEF1E1, WRN, PGGT1B, SOD2

Articles related to progeria:

(show top 50)    (show all 70)

id	Title	Authors	Year	Affiliating Genes
1	Different prelamin A forms accumulate in human fibrob lasts: a study in experimental models and progeria. (19351612)	Dominici S.... Lattanzi G.	2009	LMNA, ZMPSTE24
2	Homozygous LMNA mutation R527C in atypical Hutchinson -Gilford progeria syndrome: evidence for autosomal recessive inheritance. (19432833)	Liang L.... Gu X.	2009	LMNA
3	Telomere length in Hutchinson-Gilford progeria syndrome. (19428457)	Decker M.L.... Lansdorp P.M.	2009	LMNA
4	Molecular ageing in progeroid syndromes: Hutchinson-Gilford progeria syndrome as a model. (19379495)	Coutinho H.D.... da NA^brega R.B.	2009	ZMPSTE24
5	Activating the synthesis of progerin, the mutant prel amin A in Hutchinson-Gilford progeria syndrome, with antisense oligonucleotides . (19376814)	Fong L.G.... Young S.G.	2009	LMNA
6	Model of human aging: recent findings on Werner's and Hutchinson-Gilford progeria syndromes. (18982914)	Ding S.L.... Shen C.Y.	2008	LMNA, WRN
7	Severe mandibuloacral dysplasia-associated lipodystrophy and progeria in a young girl with a novel homozygous Arg527Cys LMNA mutation. (18796515)	Agarwal A.K.... Garg A.	2008	LMNA, ZMPSTE24
8	Epidermal expression of the truncated prelamin A causing Hutchinson-Gilford progeria syndrome: effects on keratinocytes, hair and skin. (18442998)	Wang Y.... Worman H.J.	2008	LMNA
9	Gone with the Wnt/Notch: stem cells in laminopathies, progeria, and aging. (18378774)	Meshorer E.... Gruenbaum Y.	2008	ZMPSTE24
10	Association of homozygous LMNA mutation R471C with new phenotype: mandibuloacral dysplasia, progeria, and rigid spine muscular dystrophy. (18348272)	Zirn B.... Hahn A.	2008	LMNA
11	Progeria caused by a rare LMNA mutation p.S143F associated with mild myopathy and atrial fibrillation. (18339564)	Madej-Pilarczyk A.... Hausmanowa-Petrusewicz I.	2008	LMNA
12	Reversible phenotype in a mouse model of Hutchinson-Gilford progeria syndrome. (18708427)	Sagelius H.... Eriksson M.	2008	LMNA
13	Nesprin-2 giant safeguards nuclear envelope architecture in LMNA S143F progeria cells. (17881656)	Kandert S.... Karakesisoglou I.	2007	LMNA, SYNE2
14	Werner's syndrome (adult onset progeria) (17375009)	Kluger N.... Aractingi S.	2007	WRN
15	Progeria of stem cells: stem cell exhaustion in Hutchinson-Gilford progeria syndrome. (17301031)	Halaschek-Wiener J.... Brooks-Wilson A.	2007	LMNA
16	A lamin A protein isoform overexpressed in Hutchinson-Gilford progeria syndrome interferes with mitosis in progeria and normal cells. (17360355)	Cao K.... Collins F.S.	2007	LMNA
17	An association of Hutchinson-Gilford progeria and malignancy. (17618517)	Shalev S.A.... Levy N.	2007	LMNA
18	The truncated prelamin A in Hutchinson-Gilford progeria syndrome alters segregation of A-type and B-type lamin homopolymers. (16481358)	Delbarre E.... Buendia B.	2006	LMNB1, SPG7
19	Hutchinson-Gilford progeria mutant lamin A primarily targets human vascular cells as detected by an anti-Lamin A G608G antibody. (16461887)	McClintock D.... Djabali K.	2006	LMNA
20	Compound heterozygosity for mutations in LMNA causes a progeria syndrome without prelamin A accumulation. (16825282)	Verstraeten V.L.... van den Wijngaard A.	2006	LMNA
21	Aggrecan expression is substantially and abnormally upregulated in Hutchinson-Gilford Progeria Syndrome dermal fibroblasts. (16650460)	Lemire J.M.... Weiss A.S.	2006	ACAN
22	Ankyrin G overexpression in Hutchinson-Gilford progeria syndrome fibroblasts identified through biological filtering of expression profiles. (17033732)	Wang J.... Hegele R.A.	2006	ANK3
23	A homozygous ZMPSTE24 null mutation in combination wi th a heterozygous mutation in the LMNA gene causes Hutchinson-Gilford progeria syndrome (HGPS): insights into the pathophysiology of HGPS. (16671095)	Denecke J.... Marquardt T.	2006	LMNA, ZMPSTE24
24	Aging: progeria and the lamin connection. (16920618)	Kudlow B.A.... Kennedy B.K.	2006	LMNA
25	Hutchinson-Gilford progeria in the light of contemporary genetics (17028399)	Madej-Pilarczyk A.	2006	LMNA
26	Rescue of progeria in trichothiodystrophy by homozygo us lethal Xpd alleles. (17020410)	Andressoo J.O.... Mitchell J.R.	2006	HELLS
27	A-type nuclear lamins, progerias and other degenerative disorders. (15722103)	Smith E.D.... Kennedy B.K.	2005	LMNA
28	Inhibiting farnesylation of progerin prevents the characteristic nuclear blebbing of Hutchinson-Gilford progeria syndrome. (16129833)	Capell B.C.... Collins F.S.	2005	LMNA
29	Dermal fibroblasts in Hutchinson-Gilford progeria syndrome with the lamin A G608G mutation have dysmorphic nuclei and are hypersensitive to heat stress. (15982412)	Paradisi M.... Djabali K.	2005	LMNA
30	Somatic and gonadal mosaicism in Hutchinson-Gilford progeria. (15793835)	Wuyts W.... Storm K.	2005	LMNA
31	Incomplete processing of mutant lamin A in Hutchinson-Gilford progeria leads to nuclear abnormalities, which are reversed by farnesyltransferase inhibition. (16126733)	Glynn M.W.... Glover T.W.	2005	LMNA
32	Reversal of the cellular phenotype in the premature aging disease Hutchinson-Gilford progeria syndrome. (15750600)	Scaffidi P.... Misteli T.	2005	LMNA
33	Correction of cellular phenotypes of Hutchinson-Gilford Progeria cells by RNA interference. (16208517)	Huang S.... Oshima J.	2005	LMNA
34	Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation. (16014412)	Yang S.H.... Fong L.G.	2005	LMNA
35	Mutations in the mouse Lmna gene causing progeria, muscular dystrophy and cardiomyopathy. (15773758)	Kozlov S.... Stewart C.L.	2005	LMNA
36	Inhibiting farnesylation reverses the nuclear morphology defect in a HeLa cell model for Hutchinson-Gilford progeria syndrome. (16186497)	Mallampalli M.P.... Michaelis S.	2005	LMNA
37	RNAi of FACE1 protease results in growth inhibition of human cells expressing lamin A: implications for Hutchinson-Gilford progeria syndrome. (15671064)	Gruber J.... Weber K.	2005	ZMPSTE24
38	Accumulation of mutant lamin A causes progressive changes in nuclear architecture in Hutchinson-Gilford progeria syndrome. (15184648)	Goldman R.D.... Collins F.S.	2004	LMNA
39	LMNA mutation in a 45 year old Japanese subject with Hutchinson-Gilford progeria syndrome. (15121795)	Fukuchi K.... Ogihara T.	2004	LMNA
40	Paternal origin of LMNA mutations in Hutchinson-Gilford progeria. (15032975)	D'Apice M.R.... Novelli G.	2004	LMNA
41	Hutchinson-Gilford progeria syndrome. (15479179)	Pollex R.L.... Hegele R.A.	2004	LMNA, ZMPSTE24
42	Recurrent de novo point mutations in lamin A cause Hutchinson-Gilford progeria syndrome. (12714972)	Eriksson M.... Collins F.S.	2003	LMNA
43	LMNA is mutated in Hutchinson-Gilford progeria (MIM 176670) but not in Wiedemann-Rautenstrauch progeroid syndrome (MIM 264090). (12768443)	Cao H.... Hegele R.A.	2003	LMNA
44	Lamin a truncation in Hutchinson-Gilford progeria. (12702809)	De Sandre-Giovannoli A.... Levy N.	2003	LMNA
45	PRELP, collagen, and a theory of Hutchinson-Gilford progeria. (12437997)	Lewis M.	2003	PRELP, NLRP12
46	A novel b(1-4)galactosyltransferase gene silent mutation (594C>T) associated with Hutchinson-Gilford progeria. (11295846)	O'Brien M.E.... Weiss A.S.	2001	B4GALT1
47	Altered levels of primary antioxidant enzymes in progeria skin fibroblasts. (10092527)	Yan T.... Oberley L.W.	1999	SOD2
48	A ScaI RFLP at the E-selectin (SELE) locus in a progeria family. (7689014)	Warnecke P.... Weiss A.S.	1993	SELE
49	Hutchinson-Gilford Progeria Syndr ome (20301300)	Gordon L.B.... Collins F.S.	1993	LMNA
50	Altered response of progeria fibroblasts to epidermal growth factor. (1808212)	Colige A.... Lapiere C.M.	1991	ELN

Genes related to progeria according to GeneCards:

(show all 39)

id	Symbol	Description	Score	GeneCards Section Context
1	LMNA	lamin A/C	11.1	Publications, Summaries, Aliases & Descriptions
2	PRELP	proline/arginine-rich end leucine-rich repeat protein	11.1	Summaries, Publications
3	BANF1	barrier to autointegration factor 1	11.0	Publications
4	ZMPSTE24	zinc metallopeptidase STE24 homolog (S. cerevisiae)	11.0	Publications
5	LMNB1	lamin B1	11.0	Publications
6	HELLS	helicase, lymphoid-specific	11.0	Publications
7	WRN	Werner syndrome, RecQ helicase-like	11.0	Publications
8	ELN	elastin	11.0	Publications
9	SOD2	superoxide dismutase 2, mitochondrial	11.0	Publications
10	GGT1	gamma-glutamyltransferase 1	11.0
11	EEF1E1	eukaryotic translation elongation factor 1 epsilon 1	11.0
12	B4GALT3	UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 3	11.0
13	TAF10	TAF10 RNA polymerase II, TATA box binding protein (TBP)-associated factor, 30kDa	11.0
14	TWIST2	twist homolog 2 (Drosophila)	11.0	Publications
15	PCGF2	polycomb group ring finger 2	11.0
16	GTF2H4	general transcription factor IIH, polypeptide 4, 52kDa	11.0
17	EMD	emerin	11.0
18	B4GALT1	UDP-Gal:betaGlcNAc beta 1,4- galactosyltransferase, polypeptide 1	11.0	Publications
19	PGGT1B	protein geranylgeranyltransferase type I, beta subunit	11.0
20	LMNB2	lamin B2	11.0
21	SUN1	Sad1 and UNC84 domain containing 1	11.0
22	XPA	xeroderma pigmentosum, complementation group A	11.0	Publications
23	SPG7	spastic paraplegia 7 (pure and complicated autosomal recessive)	11.0	Publications
24	NLRP12	NLR family, pyrin domain containing 12	11.0	Publications
25	ACAN	aggrecan	11.0	Publications
26	TRDMT1	tRNA aspartic acid methyltransferase 1	11.0
27	ENPP1	ectonucleotide pyrophosphatase/phosphodiesterase 1	11.0	Publications
28	ERCC6	excision repair cross-complementing rodent repair deficiency, complementation group 6	11.0
29	SRSF6	serine/arginine-rich splicing factor 6	11.0
30	ERCC4	excision repair cross-complementing rodent repair deficiency, complementation group 4	11.0
31	RNF130	ring finger protein 130	11.0
32	TNFRSF11B	tumor necrosis factor receptor superfamily, member 11b	11.0	Publications
33	ERCC1	excision repair cross-complementing rodent repair deficiency, complementation group 1 (includes overlapping antisense sequence)	11.0
34	SYNE2	spectrin repeat containing, nuclear envelope 2	11.0	Publications
35	ADIPOQ	adiponectin, C1Q and collagen domain containing	11.0	Publications
36	ANK3	ankyrin 3, node of Ranvier (ankyrin G)	11.0	Publications
37	SELE	selectin E	11.0	Publications
38	INS	insulin	11.0	Publications
39	TP53	tumor protein p53	11.0	Publications

Pathways related to progeria according to GeneDecks:

id	Pathway	Score	Top Affiliating Genes
1	Granzyme Pathway36	10.1	LMNA, LMNB1, LMNB2
2	Apoptosis and survival_Caspase cascade41	10.1	LMNB2, LMNB1, LMNA
3	Apoptosis and survival Caspase cascade10	10.0	LMNB2, LMNB1, LMNA
4	MPS IIIC - Sanfilippo syndrome C38	9.9	PRELP, ACAN, B4GALT3, B4GALT1
5	Dual incision reaction in GG-NER38	9.8	ERCC4, ERCC1, XPA
6	Nucleotide Excision Repair Pathway36	9.5	ERCC6, ERCC4, ERCC1, XPA
7	Cell adhesion_Cell-matrix glycoconjugates41	9.5	SELE, ACAN, ELN
8	Nucleotide excision repair20	9.4	XPA, GTF2H4, ERCC1, ERCC4, ERCC6
9	Cell adhesion Cell-matrix glycoconjugates10	9.2	SELE, ACAN, ELN
10	DNA Repair Mechanisms36	9.0	XPA, ERCC1, ERCC4, ERCC6, TP53

Compounds related to progeria according to GeneDecks:

(show all 41)

id	Compound	Score	Top Affiliating Genes
1	8-hydroxyadenine32	10.4	WRN, HELLS, ERCC6
2	8-hydroxyguanine32	10.4	SOD2, HELLS, ERCC6
3	keratan sulfate32	10.4	ACAN, ELN, PRELP
4	n-acetyllactosamine32 18	11.3	B4GALT1, B4GALT3
5	8-oxoguanine32	10.3	WRN, SOD2, HELLS, ERCC6
6	ascorbic acid32 18	11.1	SOD2, ACAN, TNFRSF11B
7	cysteine32	10.1	WRN, HELLS, NLRP12, LMNB1
8	psoralen32	10.0	XPA, ERCC1, ERCC4
9	procollagen32	10.0	ELN, ACAN, TNFRSF11B, ZMPSTE24
10	bleomycin32 9 9	12.0	TP53, SOD2, WRN
11	amifostine32 9 9	12.0	SOD2, TP53, ENPP1
12	n-acetoxy-2-acetylaminofluorene32	9.8	TP53, ERCC1
13	bcnu32	9.7	ERCC1, TP53, SOD2
14	benzo(a)pyrene32	9.6	XPA, ERCC1, TP53, HELLS
15	heparin32 9 18 9	12.6	ADIPOQ, SOD2, PRELP, ELN, TNFRSF11B
16	oxaliplatin32 34 9 9	12.6	XPA, ERCC1, TP53, GGT1
17	nash32	9.4	GGT1, ADIPOQ, INS
18	mitomycin c32	9.3	SOD2, TP53, ERCC4, ERCC1, XPA
19	vitamin d32	9.3	ADIPOQ, SOD2, ELN, ACAN, SPG7, TNFRSF11B
20	glutamate32	9.3	HELLS, ACAN, NLRP12, SPG7, INS
21	vitamin a32 9 18 9	12.2	GGT1, ADIPOQ, SOD2, ELN, SELE
22	proline32	9.2	PRELP, HELLS, ELN, ACAN, NLRP12, TP53
23	ly29400232	9.2	ADIPOQ, SOD2, TNFRSF11B, SELE
24	metformin32 34 9 9	12.2	GGT1, ADIPOQ, ENPP1, INS
25	thymidylate32	9.2	HELLS, TP53, ERCC4, ERCC1
26	atp32	9.0	WRN, SOD2, HELLS, TNFRSF11B, ERCC6, ENPP1
27	cisplatin32 34 9 9	12.0	GGT1, WRN, SOD2, HELLS, TP53, ERCC4
28	h2o232	9.0	GGT1, ADIPOQ, WRN, SOD2, HELLS, ELN
29	gliclazide32 34 9 9	11.9	SELE, INS, ADIPOQ
30	methotrexate32 34 42 9 9	12.8	GGT1, ADIPOQ, SOD2, TNFRSF11B, ERCC1, SELE
31	glycerol32 9 18 9	11.8	ADIPOQ, HELLS, TRDMT1, ERCC1, INS
32	5fluorouracil32	8.7	SOD2, TP53, ERCC4, ERCC1, SELE
33	doxorubicin32 34 9 9	11.7	SELE, ERCC1, TP53, TNFRSF11B, HELLS, SOD2
34	rosiglitazone32 9 18 9	11.5	SELE, LMNA, INS, ADIPOQ
35	8-isoprostane32	8.5	SELE, INS, ADIPOQ
36	etoposide32 42 9 9	11.4	SOD2, TP53, ERCC1, LMNB1, SELE
37	dexamethasone32 42 34 9 9	12.3	GGT1, ADIPOQ, INS, ENPP1, TNFRSF11B, ACAN
38	alanine32	8.1	GGT1, ADIPOQ, SOD2, HELLS, ELN, INS
39	arginine32	8.1	GGT1, ADIPOQ, WRN, SOD2, PRELP, HELLS
40	creatinine32	7.6	SELE, GGT1, ADIPOQ, SOD2, ELN, SPG7
41	serine32	6.4	GGT1, ADIPOQ, HELLS, ELN, NLRP12, TNFRSF11B

Cellular components related to progeria according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	nuclear lamina	GO:005652	10.2	LMNA, LMNB2
2	lamin filament	GO:005638	10.1	LMNB2, LMNB1, LMNA
3	nuclear inner membrane	GO:005637	9.8	LMNB1, LMNB2, SUN1, EMD
4	nucleotide-excision repair complex	GO:000109	9.5	ERCC1, ERCC4
5	nuclear envelope	GO:005635	9.4	EMD, SYNE2, SUN1, LMNB1, LMNA
6	nucleoplasm	GO:005654	7.5	LMNA, WRN, SYNE2, TAF10, SRSF6, TP53

Biological processes related to progeria according to GeneDecks:

(show all 19)

id	Name	GO ID	Score	Top Affiliating Genes
1	response to superoxide	GO:000303	10.1	SOD2, ERCC6
2	keratan sulfate biosynthetic process	GO:018146	10.1	B4GALT1, B4GALT3, ACAN, PRELP
3	keratan sulfate metabolic process	GO:042339	10.1	B4GALT1, B4GALT3, ACAN, PRELP
4	positive regulation of cell aging	GO:090343	10.1	TP53, LMNA
5	replicative cell aging	GO:001302	10.1	WRN, ERCC1
6	nuclear envelope organization	GO:006998	10.1	SYNE2, SUN1, ZMPSTE24
7	nucleotide-excision repair, DNA incision, 5-to lesion	GO:006296	10.0	ERCC4, ERCC1
8	nucleotide-excision repair, DNA incision, 3-to lesion	GO:006295	10.0	ERCC4, ERCC1
9	cell aging	GO:007569	10.0	TP53, PRELP, WRN
10	negative regulation of telomere maintenance	GO:032205	10.0	ERCC4, ERCC1
11	negative regulation of protein autophosphorylation	GO:031953	9.8	ADIPOQ, NLRP12, ENPP1
12	nucleotide-excision repair, DNA damage removal	GO:000718	9.8	ERCC4, ERCC1, GTF2H4, XPA
13	glycosaminoglycan metabolic process	GO:030203	9.7	PRELP, ACAN, B4GALT3, B4GALT1
14	response to UV	GO:009411	9.7	XPA, ERCC4, ERCC6
15	response to oxidative stress	GO:006979	9.7	XPA, ERCC1, ERCC6, WRN
16	transcription-coupled nucleotide-excision repair	GO:006283	9.6	GTF2H4, ERCC1, ERCC4, ERCC6
17	cytoskeletal anchoring at nuclear membrane	GO:090286	9.6	SUN1, SYNE2
18	base-excision repair	GO:006284	9.6	ERCC6, TP53, WRN
19	nucleotide-excision repair	GO:006289	9.0	XPA, GTF2H4, ERCC1, ERCC4, ERCC6, TP53

Molecular functions related to progeria according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	beta-N-acetylglucosaminylglycopeptide beta-1,4-galactosyltransferase activity	GO:003831	10.2	B4GALT1, B4GALT3
2	N-acetyllactosamine synthase activity	GO:003945	10.1	B4GALT3, B4GALT1
3	damaged DNA binding	GO:003684	9.6	XPA, ERCC1, ERCC4
4	structure-specific DNA binding	GO:043566	9.5	ERCC1, ERCC4
5	protein binding	GO:005515	3.4	PCGF2, NLRP12, ACAN, ELN, TWIST2, TAF10