Wolfram Syndrome malady

Genetics Home Reference: Wolfram syndrome is a condition that affects many of the body's systems. The hallmark features of Wolfram syndrome are high blood sugar levels resulting from a shortage of the hormone insulin (diabetes mellitus) and progressive vision loss due to degeneration of the nerves that carry information from the eyes to the brain (optic atrophy). People with Wolfram syndrome often also have pituitary gland dysfunction that results in the excretion of excessive amounts of urine (diabetes insipidus), hearing loss caused by changes in the inner ear (sensorineural deafness), urinary tract problems, reduced amounts of the sex hormone testosterone in males (hypogonadism), or neurological or psychiatric disorders.¹⁷

MalaCards: Wolfram Syndrome, also known as didmoad, is related to type 1 diabetes mellitus and wolfram syndrome 2. An important gene associated with Wolfram Syndrome is WFS1 (Wolfram syndrome 1 (wolframin)), and among its related pathways are GABA signaling in brain and Mechanisms of CFTR activation by S-nitrosoglutathione (normal and CF). The drug chromium picolinate and the compounds tungsten and " l-655,708 " have been mentioned in the context of this disorder. Affiliated tissues include brain, t cells and b cells, and related mouse phenotypes are skeleton and liver/biliary system.

Disease Ontology: A genetic disease that is characterized by diabetes mellitus, optic atrophy, and deafness as well as various other possible disorders and has material basis in autosomal recessive inheritance of homozygous or compound heterozygous mutation in the gene encoding wolframin (wfs1).⁶

NIH Rare Diseases: Wolfram syndrome, which is also known by the acronym DIDMOAD, is characterized by diabetes insipidus (DI); childhood-onset diabetes mellitus (DM); a gradual loss of vision caused by optic atrophy (OA); and deafness (D). Other complications that affect the bladder and nervous system may also occur. Mutations in two genes have been associated with Wolfram syndrome. The majority of cases are associated with mutations in the WFS1 gene. Mutations in the CISD2 gene have been identified in three consanguineous families of Jordanian descent. The condition is inherited in an autosomal recessive manner. Treatment is symptomatic and supportive.³⁰

Wikipedia: Wolfram syndrome, also called DIDMOAD (Diabetes Insipidus, Diabetes Mellitus, Optic Atrophy, and...⁴⁴ more...

OMIM: 222300

Aliases & Descriptions:

wolfram syndrome 6 7 30 16 17 8 33 32 43 didmoad 6 30 17 diabetes insipidus and mellitus with optic atrophy and deafness 30 17 didmoad syndrome 30 16 diabetes mellitus and insipidus with optic atrophy and deafness (disorder) 6

diabetes insipidus, diabetes mellitus, optic atrophy, and deafness 17 central diabetes insipidus 43 diabetes mellitus 43 wfs 30

Disease types for wolfram syndrome family:

wolfram syndrome 2

Diseases related to wolfram syndrome by text searches and GeneDecks gene sharing:

(show top 50)    (show all 418)

id	Related Disease	Score	Top Affiliating Genes
1	type 1 diabetes mellitus	38.5	COMT, HLA-DRB1, INS, WFS1
2	wolfram syndrome 2	32.7	CISD1, CISD2, TOMM40, WFS1
3	celiac disease	31.7	HLA-DRB1, HLA-DRB3, GABRA4, INS, SCG5
4	optic atrophy	30.7	WFS1, NPS, OPA3, BAMBI, CISD2, RECK
5	hearing loss	29.9	OPA3, COMT, TH, DIAPH1, HLA-DRB1, WFS1
6	diabetes insipidus	29.7	SCG5, CISD2, WFS1, INS
7	cholesterol	29.3	UBC, GABRA4, GABRA2, TH, TOMM40, ATF6
8	sensorineural hearing loss	29.3	HLA-DRB1, WFS1, DIAPH1, OPA3
9	dementia	28.7	TH, TOMM40, COMT, SP1, HLA-DRB3, HLA-DRB1
10	cystic fibrosis	28.7	SP3, SP1, INS, ATF6, UBC, CISD1
11	ischemia	28.3	ATF6, UBC, INS, HSPA5, SP1, COMT
12	bipolar affective disorder	28.1	COMT, WFS1, TH, DRD5
13	twinning	28.1	COMT, GABRA2, EFEMP1, TH, SP1, HLA-DRB1
14	neurodegenerative disease	28.0	BAMBI, HSPA5, RECK, COMT, WFS1, SYVN1
15	rheumatoid arthritis	27.9	HLA-DRB1, SYVN1, HSPA5, INS, UBC, RECK
16	diabetes mellitus	27.8	INS, HLA-DRB1, HLA-DRB3, COMT, WFS1, HSPA5
17	multiple endocrine neoplasia	27.8	SP1, TH, INS, UBC
18	cerebritis	27.6	SMURF1, GABRA4, COMT, DRD5, HLA-DRB1, HSPA5
19	hepatitis c	27.3	COMT, SP1, HLA-DRB1, HSPA5, INS, HLA-DRB3
20	arthritis	27.3	SYVN1, TH, RECK, SCG5, HLA-DRB3, UBC
21	retinitis	27.1	SP3, INS, BAMBI, UBC, SEMA3E, HLA-DRB1
22	gestational diabetes	27.0	HLA-DRB1, INS, HLA-DRB3, WFS1
23	immunodeficiency	26.4	PSMA5, HLA-DRB1, RECK, SCG5, UBC, INS
24	bipolar disorder	25.8	ATF6, GABRA2, HSPA5, HLA-DRB1, DRD5, COMT
25	huntington's disease	25.8	ATF6, TH, SP1, DRD5, UBC, SP3
26	alzheimer's disease	25.7	CISD1, UBC, ATF6, INS, HSPA5, DRD5
27	ataxia	25.6	WFS1, SYVN1, SP1, HSPA5, EFEMP1, INS
28	parkinson's disease	24.9	DRD5, HLA-DRB1, INS, UBC, HSPA5, TH
29	adenoma	24.7	RECK, SCG5, COMT, SP1, INS, TH
30	pancreatitis	23.5	HSPA5, INHBE, COMT, INS, SMURF1, SP3
31	periodontitis	23.4	SEMA3D, SP1, INS, HSPA5, HLA-DRB1, HLA-DRB3
32	schizophrenia	23.3	SP1, DRD5, HLA-DRB1, HSPA5, INS, UBC
33	hepatocellular carcinoma	22.9	TH, RECK, UBC, HSPA5, COMT, SP3
34	fibrosis	22.7	SP1, SP3, RECK, HLA-DRB1, INS, SCG5
35	neuronitis	20.8	DRD5, SEMA3D, UBC, ATF6, INHBE, INS
36	prostatitis	20.3	ATF6, COMT, EFEMP1, WFS1, RECK, HLA-DRB1
37	type 2 diabetes mellitus	15.1
38	opiate dependence	13.0	COMT, TH
39	substance dependence	12.8	DRD5, COMT, TH
40	retinal disease	12.8	BAMBI, INS, EFEMP1, OPA3
41	cervical dystonia	12.6	HLA-DRB1, DRD5, TH
42	nicotine dependence	12.4	COMT, GABRA2, TH, DRD5, GABRA4
43	schizoaffective disorder	12.4	INS, COMT, GABRA2, GABRG1
44	alcohol dependence	12.3	COMT, GABRA2, TH, GABRG1, GABRA4
45	immunodysregulation	12.2	HLA-DRB1, INS
46	hypertension	12.2
47	nephropathy	12.2
48	oculomotor apraxia cogan type	12.0	COMT, UBC, INS, DRD5, TH
49	amyotrophic lateral sclerosis	11.9	TOMM40, TH, COMT, HSPA5, UBC, EFEMP1
50	lateral sclerosis	11.9	UBC, TH, HSPA5, TOMM40, COMT, EFEMP1

Clinical features from OMIM: 222300

Approved drugs:

Drug clinical trials:

Inferred drug relations via UMLS/NDF-RT:

^43 28 chromium picolinate

Genetic tests related to wolfram syndrome:

id	Genetic test	Affiliating Genes
1	Wolfram Syndrome clinical/research	WFS1

MalaCards organs/tissues related to wolfram syndrome:

²²

MGI Mouse Phenotypes related to wolfram syndrome:

²⁵

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	skeleton phenotype	MP:0005390	8.1	SYVN1, SP3, SP1, DIAPH1, INS, BAMBI
2	liver/biliary system phenotype	MP:0005370	7.2	RECK, SCG5, UBC, ATF6, INS, SP1
3	vision/eye phenotype	MP:0005391	7.2	EFEMP1, OPA3, TH, SP3, SP1, DRD5
4	nervous system phenotype	MP:0003631	7.0	TH, COMT, SMURF1, DRD5, INS, CISD2
5	behavior/neurological phenotype	MP:0005386	6.5	GABRA4, GABRA2, WFS1, OPA3, TH, COMT
6	integument phenotype	MP:0010771	6.4	RECK, GABRA4, GABRA2, EFEMP1, WFS1, TH
7	growth/size phenotype	MP:0005378	6.2	SP1, SP3, TH, OPA3, WFS1, EFEMP1
8	cardiovascular system phenotype	MP:0005385	6.1	EFEMP1, OPA3, TH, RECK, SEMA3E, SCG5
9	mortality/aging	MP:0010768	5.8	SMURF1, TH, SYVN1, OPA3, WFS1, EFEMP1
10	homeostasis/metabolism phenotype	MP:0005376	5.6	COMT, TH, OPA3, WFS1, EFEMP1, GABRA2

Articles related to wolfram syndrome:

(show top 50)    (show all 63)

id	Title	Authors	Year	Affiliating Genes
1	Atypical case of Wolfram syndrome revealed through targeted exome sequencing in a patient with suspected mitochondrial disease. (22226368)	Lieber D.S.... Mootha V.K.	2012	WFS1
2	The E3 ligase Smurf1 regulates Wolfram syndrome prote in stability at the endoplasmic reticulum. (21454619)	Guo X.... Zhang L.	2011	UBC, SMURF1, ATF6
3	Wolfram syndrome: clinical and genetic analysis in t wo sisters]. (21632151)	Conart J.B.... Angioi K.	2011	WFS1
4	An evaluation of HLA class 2 alleles and anti-islet antibodies as evidence for non-autoimmune diabetes in Wolfram syndrome]. (21447263)	ZmysA8owska A.... MA8ynarski W.	2010	HLA-DRB1, HLA-DRB3
5	Wolfram syndrome 1 (Wfs1) mRNA expression in the normal mouse brain during postnatal development. (19428703)	Kawano J.... Shinoda K.	2009	WFS1
6	Identification of novel mutations of the WFS1 gene in Brazilian patients with Wolfram syndrome. (19042979)	Gasparin M.R.... MoisAcs R.S.	2009	WFS1
7	Current developments in Wolfram syndrome. (19344068)	Ganie M.A.... Bhat D.	2009	WFS1
8	The novel compound heterozygous mutations, V434del and W666X, in WFS1 gene causing the Wolfram syndrome in a Chinese family. (19160074)	Hong J.... Ning G.	2009	WFS1
9	Wolfram syndrome. Clinical and genetic study in two families (18194629)	Lou Frances G.... Rodriguez Rigual M.	2008	WFS1
10	Distribution of Wfs1 protein in the central nervous system of the mouse and its relation to clinical symptoms of the Wolfram syndrome. (18551525)	Luuk H.... Vasar E.	2008	WFS1
11	Wolfram syndrome (diabetes insipidus, diabetes, optic atrophy, and deafness): clinical and genetic study. (18566338)	d'Annunzio G.... Lorini R.	2008	WFS1
12	Hearing impairment in genotyped Wolfram syndrome patients. (18700423)	Plantinga R.F.... Cremers C.W.	2008	WFS1
13	Familial Wolfram syndrome due to compound heterozygosity for two novel WFS1 mutations. (18660851)	Zenteno J.C.... Camargo M.	2008	WFS1
14	Identification of novel mutations in WFS1 and genotype-phenotype correlation in Wolfram syndrome. (17568405)	Cano A.... Vialettes B.	2007	WFS1
15	A homozygous mutation in a novel zinc-finger protein, ERIS, is responsible for Wolfram syndrome 2. (17846994)	Amr S.... Shiang R.	2007	WFS1, CISD2
16	The characterisation of the human Wolfram syndrome gene promoter demonstrating regulation by Sp1 and Sp3 transcription factors. (16965966)	Ricketts C.... Barrett T.	2006	SP1, WFS1, SP3
17	A new mutation in WFS1 gene (C.1522-1523delTA, Y508fsX421) may be responsible for early appearance of clinical features of Wolfram syndrome and suicidal behaviour. (17187023)	Aluclu M.U.... Gokalp D.	2006	WFS1
18	A novel mutation of WFS1 gene in a Japanese man of Wolfram syndrome with positive diabetes-related antibodies. (16442662)	Nakamura A.... Koike T.	2006	WFS1
19	Mutation analysis of the WFS1 gene in seven Danish Wolfram syndrome families; four new mutations identified. (16151413)	Hansen L.... Rosenberg T.	2005	WFS1
20	Identification of a novel WFS1 mutation (AFF344-345ins) in Japanese patients with Wolfram syndrome. (16005363)	Inukai K.... Katayama S.	2005	WFS1
21	Wolfram syndrome in French population: characterization of novel mutations and polymorphisms in the WFS1 gene. (15605410)	Giuliano F.... Paquis-Flucklinger V.	2005	WFS1
22	A novel mutation of WFS1 gene in Chinese patients with Wolfram syndrome (16321270)	Fang Q.C.... Xiang K.S.	2005	WFS1
23	Expressional and functional studies of Wolframin, the gene function deficient in Wolfram syndrome, in mice and patient cells. (16087305)	Philbrook C.... Weiher H.	2005	EFEMP1, WFS1
24	Heterogenous mutations of Wolfram syndrome I gene responsible for low frequency nonsyndromic hearing loss (16408729)	Liu Y.H.... Xiao S.F.	2005	WFS1
25	Clinical picture, evolution and peculiar molecular fi ndings in a very large pedigree with Wolfram syndrome. (16459465)	Lombardo F.... De Luca F.	2005	WFS1
26	A mutation in Wolfram syndrome type 1 gene in a Japanese family with autosomal dominant low-frequency sensorineural hearing loss. (16353398)	Noguchi Y.... Kitamura K.	2005	WFS1
27	Two families with nonsyndromic low-frequency hearing loss harbor novel mutations in Wolfram syndrome gene 1. (15912360)	Gurtler N.... Lalwani A.K.	2005	WFS1
28	Phenotype-genotype correlations in a series of wolfram syndrome families. (15277431)	Smith C.J.... Scott R.J.	2004	WFS1
29	Study of the WFS1 gene and mitochondrial DNA in Spanish Wolfram syndrome families. (15151504)	Domenech E.... Nunes V.	2004	WFS1
30	Genetic diagnosis of diabetes mellitus: Wolfram syndrome--from positional cloning to DNA diagnosis (12884741)	Tanizawa Y.	2003	WFS1, BAMBI
31	Molecular detection of novel WFS1 mutations in patients with Wolfram syndrome by a DHPLC-based assay. (12754709)	Colosimo A.... Dallapiccola B.	2003	WFS1
32	Mutations in the Wolfram syndrome type 1 gene (WFS1) define a clinical entity of dominant low-frequency sensorineural hearing loss. (12707187)	Lesperance M.M.... Leal S.M.	2003	WFS1
33	Mutational spectrum of the WFS1 gene in Wolfram syndrome, nonsyndromic hearing impairment, diabetes mellitus, and psychiatric disease. (12955714)	Cryns K.... Van Camp G.	2003	WFS1
34	Multicentre Italian family-based association study on tyrosine hydroxylase, catechol-O-methyl transferase and Wolfram syndrome 1 polymorphisms in mood disorders. (12782971)	Serretti A.... Nappi G.	2003	COMT, TH, WFS1
35	The WFS1 (Wolfram syndrome 1) is not a major suscepti bility gene for the development of psychiatric disorders. (12605098)	Martorell L.... Nunes V.	2003	WFS1
36	Wolfram syndrome and suicide: Evidence for a role of WFS1 in suicidal and impulsive behavior. (12707947)	Sequeira A.... Turecki G.	2003	WFS1
37	Wolfram syndrome: phenotype and novel mutation in two Taiwanese siblings. (14724730)	Shu S.G.... Chi C.S.	2003	WFS1, CISD2
38	Wolfram syndrome: structural and functional analyses of mutant and wild-type wolframin, the WFS1 gene product. (12913071)	Hofmann S.... Bauer M.F.	2003	WFS1
39	Bleeding tendency in Wolfram syndrome: a newly identified feature with phenotype genotype correlation. (11317648)	l-Sheyyab M.... Ajlouni K.	2001	CISD2
40	Mutations in the Wolfram syndrome 1 gene (WFS1) are a common cause of low frequency sensorineural hearing loss. (11709537)	Bespalova I.N.... Lesperance M.M.	2001	WFS1
41	Non-syndromic progressive hearing loss DFNA38 is caused by heterozygous missense mutation in the Wolfram syndrome gene WFS1. (11709538)	Young T.-L.... King M.-C.	2001	WFS1
42	WFS1 (Wolfram syndrome 1) gene product: predominant subcellular localization to endoplasmic reticulum in cultured cells and neuronal expression in rat brain. (11181571)	Takeda K.... Oka Y.	2001	HSPA5, WFS1
43	Mutational analysis of the Wolfram syndrome gene in two families with chromosome 4p-linked bipolar affective disorder. (10893488)	Evans K.L.... Porteous D.J.	2000	WFS1
44	Missense variations of the gene responsible for Wolfram syndrome (WFS1/wolframin) in Japanese: possible contribution of the Arg456His mutation to type 1 diabetes as a nonautoimmune genetic basis. (10679252)	Awata T.... Katayama S.	2000	WFS1, BAMBI
45	Homozygosity mapping identifies an additional locus for Wolfram syndrome on chromosome 4q. (10739754)	El-Shanti H.... Ajlouni K.	2000	BAMBI, CISD2
46	Positional cloning of the gene(WFS1) for Wolfram syndrome (11215108)	Tanizawa Y.... Oka Y.	2000	WFS1, BAMBI
47	Clinical and molecular genetic analysis of 19 Wolfram syndrome kindreds demonstrating a wide spectrum of mutations in WFS1. (10521293)	Hardy C.... Barrett T.	1999	WFS1
48	The vasopressin precursor is not processed in the hypothalamus of Wolfram syndrome patients with diabetes insipidus: evidence for the involvement of PC2 and 7B2. (9814487)	Gabreels B.A.... van Leeuwen F.W.	1998	SCG5
49	Linkage of Wolfram syndrome to chromosome 4p16.1 and evidence for heterogeneity. (8808601)	Collier D.A.... Bundey S.	1996	DRD5
50	Linkage of the gene for Wolfram syndrome to markers on the short arm of chromosome 4. (7987399)	Polymeropoulos M.H.... Swift M.	1994	WFS1

Genes related to wolfram syndrome according to GeneCards:

(show all 31)

id	Symbol	Description	Score	GeneCards Section Context
1	WFS1	Wolfram syndrome 1 (wolframin)	11.1	Functions, Publications, Aliases & Descriptions, Summaries (show all 6 sections) Transcripts, Orthologs
2	CISD2	CDGSH iron sulfur domain 2	11.1	Summaries, Aliases & Descriptions, Publications
3	BAMBI	BMP and activin membrane-bound inhibitor homolog (Xenopus laevis)	11.0	Publications
4	CISD1	CDGSH iron sulfur domain 1	11.0	Publications
5	TOMM40	translocase of outer mitochondrial membrane 40 homolog (yeast)	11.0	Publications
6	RECK	reversion-inducing-cysteine-rich protein with kazal motifs	11.0
7	INHBE	inhibin, beta E	11.0
8	SYVN1	synovial apoptosis inhibitor 1, synoviolin	11.0	Publications
9	NPS	neuropeptide S	11.0
10	PSMA5	proteasome (prosome, macropain) subunit, alpha type, 5	11.0	Publications
11	DRD5	dopamine receptor D5	11.0	Publications
12	EFEMP1	EGF containing fibulin-like extracellular matrix protein 1	11.0	Publications
13	HLA-DRB3	major histocompatibility complex, class II, DR beta 3	11.0	Publications
14	ATF6	activating transcription factor 6	11.0	Publications
15	SP3	Sp3 transcription factor	11.0	Publications
16	GABRG1	gamma-aminobutyric acid (GABA) A receptor, gamma 1	11.0
17	GABRA4	gamma-aminobutyric acid (GABA) A receptor, alpha 4	11.0
18	OPA3	optic atrophy 3 (autosomal recessive, with chorea and spastic paraplegia)	11.0
19	SMURF1	SMAD specific E3 ubiquitin protein ligase 1	11.0	Publications
20	SCG5	secretogranin V (7B2 protein)	11.0	Publications
21	SEMA3D	sema domain, immunoglobulin domain (Ig), short basic domain, secreted, (semaphorin) 3D	11.0
22	DIAPH1	diaphanous homolog 1 (Drosophila)	11.0
23	GABRA2	gamma-aminobutyric acid (GABA) A receptor, alpha 2	11.0
24	UBC	ubiquitin C	11.0	Publications
25	HSPA5	heat shock 70kDa protein 5 (glucose-regulated protein, 78kDa)	11.0	Publications
26	TH	tyrosine hydroxylase	11.0	Publications
27	SEMA3E	sema domain, immunoglobulin domain (Ig), short basic domain, secreted, (semaphorin) 3E	11.0
28	COMT	catechol-O-methyltransferase	11.0	Publications
29	SP1	Sp1 transcription factor	11.0	Publications
30	INS	insulin	11.0	Publications
31	HLA-DRB1	major histocompatibility complex, class II, DR beta 1	11.0	Publications

Pathways related to wolfram syndrome according to GeneDecks:

id	Pathway	Score	Top Affiliating Genes
1	GABA signaling in brain10	9.4	GABRA2, GABRG1, GABRA4
2	Mechanisms of CFTR activation by S-nitrosoglutathione (normal and CF)41	9.2	HSPA5, SP1, SP3
3	Type I diabetes mellitus20	8.9	INS, HLA-DRB1, HLA-DRB3

Compounds related to wolfram syndrome according to GeneDecks:

(show all 32)

id	Compound	Score	Top Affiliating Genes
1	tungsten32 18	11.5	RECK, BAMBI, WFS1
2	l-655,708 42	10.1	GABRA4, GABRG1, GABRA2
3	sr 95531 hydrobromide42	10.1	GABRA4, GABRG1, GABRA2
4	(+)-bicuculline42	10.1	GABRA4, GABRG1, GABRA2
5	Clorazepate9 9	11.1	GABRA4, GABRG1, GABRA2
6	aa 2950442	10.1	GABRA4, GABRA2
7	Oxazepam9 9	11.1	GABRA4, GABRG1, GABRA2
8	nitrazepam32 9 9	12.1	GABRA4, GABRG1, GABRA2
9	flurazepam32 9 9	12.1	GABRA4, GABRG1, GABRA2
10	clobazam34 32 9 9	13.0	GABRA4, GABRG1, GABRA2
11	bromazepam32 9 9	12.0	GABRA4, GABRG1, GABRA2
12	Temazepam9 9	11.0	GABRA4, GABRG1, GABRA2
13	clonazepam32 9 9	12.0	GABRA4, GABRG1, GABRA2
14	lorazepam32 34 9 9	13.0	GABRA4, GABRG1, GABRA2
15	chlordiazepoxide32 9 9	12.0	GABRA2, GABRG1, GABRA4
16	muscimol32 42	11.0	GABRA4, GABRG1, GABRA2
17	triazolam32 9 9	12.0	GABRA2, GABRG1, GABRA4
18	alprazolam32 9 9	11.9	GABRA2, GABRG1, GABRA4
19	benserazide32	9.8	COMT, TH
20	nialamide32 9 9	11.8	TH, COMT
21	methylphenidate32 9 9	11.7	TH, COMT, DRD5
22	pramipexole32 9 9	11.7	DRD5, COMT, TH
23	l-dops32	9.7	TH, COMT
24	midazolam32 9 9	11.6	GABRA2, GABRG1, GABRA4
25	apomorphine32 9 9	11.6	DRD5, COMT, TH
26	tunicamycin32	9.5	RECK, ATF6, HSPA5, SP1
27	catecholamine32	9.4	TH, COMT, DRD5, SCG5
28	methionine32	9.0	COMT, SP3, HSPA5, SCG5, RECK
29	olanzapine32 34 9 18 9	12.7	COMT, DRD5, INS
30	dopamine32 9 18 9	11.3	TH, COMT, SP3, SP1, DRD5, HSPA5
31	glutamate32	7.6	SCG5, INS, SP1, SP3, COMT, TH
32	tyrosine32	6.8	TH, SP3, SP1, DRD5, HLA-DRB1, HSPA5

Cellular components related to wolfram syndrome according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	chloride channel complex	GO:034707	9.7	GABRA2, GABRG1, GABRA4
2	endocytic vesicle membrane	GO:030666	9.1	UBC, HLA-DRB1, HLA-DRB3

Biological processes related to wolfram syndrome according to GeneDecks:

(show all 16)

id	Name	GO ID	Score	Top Affiliating Genes
1	ER-associated protein catabolic process	GO:030433	10.0	WFS1, SYVN1, HSPA5
2	gamma-aminobutyric acid signaling pathway	GO:007214	10.0	GABRA2, GABRG1, GABRA4
3	regulation of interleukin-4 production	GO:032673	10.0	HLA-DRB1, HLA-DRB3
4	regulation of interleukin-10 secretion	GO:2001179	9.9	HLA-DRB1, HLA-DRB3
5	activation of signaling protein activity involved in unfolded protein response	GO:006987	9.9	WFS1, SYVN1, HSPA5, ATF6
6	immunoglobulin production involved in immunoglobulin mediated immune response	GO:002381	9.9	HLA-DRB1, HLA-DRB3
7	endoplasmic reticulum unfolded protein response	GO:030968	9.9	WFS1, SYVN1, HSPA5, ATF6
8	positive regulation of protein ubiquitination	GO:031398	9.8	WFS1, SMURF1, HSPA5
9	humoral immune response mediated by circulating immunoglobulin	GO:002455	9.8	HLA-DRB1, HLA-DRB3
10	negative regulation of NAD(P)H oxidase activity	GO:033861	9.7	INS, DRD5
11	negative regulation of transforming growth factor beta receptor signaling pathway	GO:030512	9.7	UBC, BAMBI, HSPA5, SMURF1
12	megakaryocyte differentiation	GO:030219	9.6	SP1, SP3
13	enucleate erythrocyte differentiation	GO:043353	9.5	SP1, SP3
14	embryonic camera-type eye morphogenesis	GO:048596	9.5	SP1, SP3, TH
15	embryonic process involved in female pregnancy	GO:060136	9.3	SP1, SP3
16	transforming growth factor beta receptor signaling pathway	GO:007179	9.3	SMURF1, SP1, BAMBI, UBC

Molecular functions related to wolfram syndrome according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	benzodiazepine receptor activity	GO:008503	9.9	GABRA2, GABRA4
2	GABA-A receptor activity	GO:004890	9.6	GABRA2, GABRG1, GABRA4
3	extracellular ligand-gated ion channel activity	GO:005230	9.4	GABRA2, GABRG1, GABRA4