Zellweger Syndrome malady

NIH Rare Diseases: Zellweger syndrome is the most severe form of a spectrum of conditions called Zellweger spectrum disorders. The signs and symptoms of Zellweger syndrome typically appear during the newborn period and may include poor muscle tone (hypotonia), poor feeding, seizures, hearing loss, vision loss, distinctive facial features, and skeletal abnormalities. Affected children also develop life-threatening problems in other organs and tissues, such as the liver, heart, and kidneys. Children with Zellweger syndrome usually do not survive beyond the first year of life. Zellweger syndrome is caused by mutations in any one of at least 12 genes; mutations in the PEX1 gene are the most common cause. It is inherited in an autosomal recessive manner. There is no cure for Zellweger syndrome; treatment is generally symptomatic and supportive.³⁰

MalaCards: Zellweger Syndrome, also known as zellweger syndrome (disorder), is related to zellweger syndrome spectrum and peroxisome biogenesis disorders, zellweger syndrome spectrum. An important gene associated with Zellweger Syndrome is PEX1 (peroxisomal biogenesis factor 1), and among its related pathways are Biosynthesis of unsaturated fatty acids and ABCA transporters in lipid homeostasis. The compounds 3-oxoacyl-coa and (2S,6R,10R)-Trimethyl-hendecanoyl-CoA have been mentioned in the context of this disorder. Affiliated tissues include heart, skeletal muscle and kidney, and related mouse phenotypes are liver/biliary system and behavior/neurological.

NINDS: Zellweger syndrome is one of a group of four related diseases called peroxisome biogenesis disorders (PBD).³¹

Genetics Home Reference: The Zellweger spectrum is a group of conditions that have overlapping signs and symptoms and affect many parts of the body. This group of disorders includes Zellweger syndrome, neonatal adrenoleukodystrophy (NALD), and infantile Refsum disease. These conditions were once thought to be distinct disorders but are now considered to be part of the same disease spectrum. Zellweger syndrome is the most severe form of the Zellweger spectrum, NALD is intermediate in severity, and infantile Refsum disease is the least severe form. In some cases, it can be difficult to distinguish between the three conditions that make up the Zellweger spectrum.¹⁷

Wikipedia: Zellweger syndrome, also called cerebrohepatorenal syndrome, is a rare congenital disorder,...⁴⁴ more...

OMIM: 214100

Aliases & Descriptions:

zellweger syndrome 6 7 30 31 8 33 32 43 zellweger syndrome (disorder) 6 16 cerebrohepatorenal syndrome 30 16 zellweger leukodystrophy 30 congenital iron overload 6

zws 30 chr 30 zes 16 zs 30

Disease types for zellweger syndrome family:

zellweger syndrome-2	zellweger syndrome-1
zellweger syndrome-3

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

(show top 50)    (show all 515)

id	Related Disease	Score	Top Affiliating Genes
1	zellweger syndrome spectrum	32.6	PEX1, PEX10, PEX12, PEX13, PEX14, PEX16
2	peroxisome biogenesis disorders, zellweger syndrome spectrum	32.1	PEX1, PEX10, PEX12, PEX2, PEX26, PEX6
3	d-bifunctional protein deficiency	31.1	EHHADH, HSD17B4
4	neonatal adrenoleukodystrophy	30.4	PEX1, PEX10, PEX13, PEX26, PEX5
5	infantile refsum disease	30.4	CAT, PEX1, PEX6
6	beta-ketothiolase deficiency	29.2	GALNS, PEX6, PEX2, ACAA2, ACAA1, ARSH
7	neuronal migration disorders	29.0	DCLK1, DCX, PEX2, NES
8	ketothiolase deficiency	27.9	GALNS, PEX6, PEX2, ACAA2, ARSH
9	peroxisome biogenesis disorders	26.5	PEX12, PEX7, PEX6, PEX13, PEX14, PEX16
10	peroxisomal biogenesis disorder	26.5	GNPAT, HSD17B4, CAT, PHEX, ABCD1, ABCD3
11	refsum disease	26.1	ACOX1, PEX1, PEX10, PEX11A, PEX12, PEX13
12	chondrodysplasia	25.5	PEX6, PEX7, HACL1, HADHB, PIPOX, SCP2
13	chondrodysplasia punctata	25.4	ACAA1, PEX26, PEX3, PEX5, PEX6, PEX7
14	rhizomelic chondrodysplasia punctata	24.8	PHYH, ACAA1, ACAA2, ACOX1, AGPS, PMVK
15	cholesterol	24.2	ACAA2, ACOX1, DBI, PMVK, SLC25A17, PEX1
16	adrenoleukodystrophy	22.5	SCP2, IDI1, AMACR, GNPAT, HSD17B4, CAT
17	refsum disease, infantile form	13.6	PEX26, PEX2
18	pseudoneonatal adrenoleukodystrophy	13.5	ACOX1, ACOX2
19	peroxisome biogenesis disorders (pbd)	13.4	PEX2, PEX5, PEX14, PEX13, PEX1, PEX10
20	trifunctional protein deficiency	13.3	HADHB, EHHADH
21	vlcad deficiency	13.3	EHHADH, ACOX3, ACOX1, ACOX2
22	mitochondrial trifunctional protein deficiency	13.3	HADHB, EHHADH
23	thiolase deficiency	13.3	HADHB, ACAA1, HSD17B4, AMACR
24	tyrosine-oxidase temporary deficiency	13.2	CAT, ACOX1
25	3-hydroxyacyl-coenzyme a dehydrogenase deficiency	13.2	HADHB, HADH, EHHADH
26	mulibrey nanism	13.1	PEX7, PEX5, PEX1
27	adrenomyeloneuropathy	13.0	SLC25A17, ABCD1
28	fatty acid oxidation disorders	13.0	SCP2, HSD17B4, PPARA
29	rhizomelic chondrodysplasia punctata type 2	13.0	GNPAT, PHYH, AGPS, PEX7
30	rhizomelic chondrodysplasia punctata type 1	12.8	HADHB, PEX7, ACOX1, HSD17B4, SCP2
31	hypotonia	12.7	PEX2, PEX26, PEX3, PEX5, PEX6, PEX19
32	seizures	12.7	PEX2, PEX26, PEX3, PEX5, PEX6, PEX19
33	peroxisomal disease	12.4	HACL1, HADHB, SLC25A17, ACOX1, ABCD3, GNPAT
34	cataract	12.4	PEX7, AGPS, PHYH, PHEX, CAT, GNPAT
35	fatty liver disease	12.3	DBI, ACOX1, NR1I3, PPARA, SCP2
36	leukodystrophy	12.3	GALNS, ACOX1, ABCD1, ARSH
37	protein s deficiency	11.8	AGPS, SLC25A17, EHHADH, HADH, HADHB, ACOX1
38	bilirubin metabolic disorder	11.7	HADH, ABCD1, NR1I3, PPARA, CAT, MVK
39	colon carcinoma	11.5	EHHADH, PEX5, SLC25A17, ACOX1, ABCD3, PPARA
40	peroxisome biogenesis factor	11.4	PEX6, PEX7, PEX13, PEX14, PEX16, PEX19
41	retinitis	10.6	GALNS, EHHADH, NES, PEX7, DCX, ACOX1
42	tuberculosis	9.6	ABCG4, ACAA1, ACAA2, AGPS, PEX1, PEX6
43	zellweger syndrome, complementation group g	8.0
44	roberts syndrome	7.5
45	neuronitis	7.3
46	pseudo-zellweger syndrome	7.3
47	zellweger syndrome-2	7.3
48	zellweger syndrome-1	7.3
49	zellweger syndrome, complementation group 9	7.3
50	zellweger syndrome-3	7.3

Clinical features from OMIM: 214100

Approved drugs:

Drug clinical trials:

MalaCards organs/tissues related to zellweger syndrome:

²²

MGI Mouse Phenotypes related to zellweger syndrome:

²⁵

id	Description	MGI Source Accession	Score	Top Affiliating Genes
1	liver/biliary system phenotype	MP:0005370	7.5	SCP2, HADHB, EHHADH, PEX7, PEX5, PEX2
2	behavior/neurological phenotype	MP:0005386	7.2	PEX13, PEX2, PEX5, PEX5L, PEX7, NES
3	growth/size phenotype	MP:0005378	5.9	PEX2, PEX13, PEX11B, PEX11A, PEX5, PEX7
4	nervous system phenotype	MP:0003631	5.9	PEX11B, PEX13, PEX2, PEX5, PEX5L, PEX7
5	mortality/aging	MP:0010768	5.4	PEX11A, PEX11B, PEX13, PEX14, PEX2, PEX5
6	cellular phenotype	MP:0005384	5.3	PEX11B, PEX13, PEX5, PEX7, NES, EHHADH
7	homeostasis/metabolism phenotype	MP:0005376	5.0	ACOX1, AGPS, DBI, PEX11B, PEX2, PEX5

Articles related to zellweger syndrome:

(show top 50)    (show all 57)

id	Title	Authors	Year	Affiliating Genes
1	Spectrum of PEX6 mutations in Zellweger syndrome spec trum patients. (19877282)	Ebberink M.S.... Waterham H.R.	2010	PEX6
2	Zellweger syndrome caused by PEX13 deficiency: report of two novel mutations. (19449432)	Al-Dirbashi O.Y.... Alkuraya F.S.	2009	PEX13
3	Identification of novel mutations and sequence variation in the Zellweger syndrome spectrum of peroxisome biogenesis disorders. (19105186)	Yik W.Y.... Hacia J.G.	2009	PEX1, PEX10, PEX6
4	Rational diagnostic strategy for Zellweger syndrome spectrum patients. (19142205)	Krause C.... GAortner J.	2009	PEX6
5	Identification of a novel PEX14 mutation in Zellweger syndrome. (18285423)	Huybrechts S.J.... Cassiman D.	2008	PEX14
6	Pathogenesis of peroxisomal deficiency disorders (Zellweger syndrome) may be mediated by misregulation of the GABAergic system via the diazepam binding inhibitor. (15102341)	Breitling R.	2004	DBI
7	The PEX Gene Screen: molecular diagnosis of peroxisome biogenesis disorders in the Zellweger syndrome spectrum. (15542397)	Steinberg S.... Braverman N.	2004	PEX1, PEX10, PEX6
8	Inhibition of peroxisomal functions due to oxidative imbalance induced by mistargeting of catalase to cytoplasm is restored by vitamin E treatment in skin fibroblasts from Zellweger syndrome-like patients. (15589116)	Kawada Y.... Singh I.	2004	CAT
9	The neuronal migration defect in mice with Zellweger syndrome (Pex5 knockout) is not caused by the inactivity of peroxisomal beta-oxidation. (11939592)	Baes M.... Reddy J.K.	2002	PEX5
10	A novel aberrant splicing mutation of the PEX16 gene in two patients with Zellweger syndrome. (11890679)	Shimozawa N.... Kondo N.	2002	PEX16
11	Mitochondrial alterations caused by defective peroxisomal biogenesis in a mouse model for Zellweger syndrome (PEX5 knockout mouse). (11583975)	Baumgart E.... Baes M.	2001	PEX5
12	Defective peroxisome membrane synthesis due to mutations in human PEX3 causes Zellweger syndrome, complementation group G. (10958759)	Muntau A.C.... Roscher A.A.	2000	PEX3
13	Identification of PEX3 as the gene mutated in a Zellweger syndrome patient lacking peroxisomal remnant structures. (10942428)	Shimozawa N.... Kondo N.	2000	PEX3, SLC25A17
14	Peroxisomal ghosts are intracellular structures distinct from lysosomal compartments in Zellweger syndrome: a confocal laser scanning microscopy study. (10879629)	Santos M.J.... Lazarow P.B.	2000	LAMP2
15	A novel migration-related gene product, doublecortin, in neuronal migration disorder of fetuses and infants with Zellweger syndrome. (10963364)	Qin J.... Takashima S.	2000	DCLK1, DCX, NES
16	PEX3 is the causal gene responsible for peroxisome membrane assembly-defective Zellweger syndrome of complementation group G. (10968777)	Ghaedi K.... Fujiki Y.	2000	PEX3
17	Phytanic acid alpha-oxidation in man: identification of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal enzyme with normal activity in Zellweger syndrome. (10896309)	Jansen G.A.... Wanders R.J.	2000	HACL1
18	A common PEX1 frameshift mutation in patients with disorders of peroxisome biogenesis correlates with the severe Zellweger syndrome phenotype. (10480353)	Maxwell M.A.... Crane D.I.	1999	PEX1
19	Identification of a common PEX1 mutation in Zellweger syndrome. (10447258)	Collins C.S.... Gould S.J.	1999	ABCD3, PEX1
20	Human PEX19: cDNA cloning by functional complementation, mutation analysis in a patient with Zellweger syndrome, and potential role in peroxisomal membrane assembly. (10051604)	Matsuzono Y.... Fujiki Y.	1999	PEX19
21	Unique multifunctional HSD17B4 gene product: 17beta-hydroxysteroid dehydrogenase 4 and D-3-hydroxyacyl-coenzyme A dehydrogenase/hydratase involved in Zellweger syndrome. (10343282)	de Launoit Y.... Adamski J.	1999	PPARA, HSD17B4
22	Zellweger syndrome in Saudi Arabia and its distinct features. (10047940)	l-Essa M.... Rahbeeni Z.	1999	GNPAT
23	Cholesterol biosynthesis, peroxisomes and peroxisomal disorders: mevalonate kinase is not only deficient in Zellweger syndrome but also in rhizomelic chondrodysplasia punctata. (9686383)	Wanders R.J.... Romeijn G.J.	1998	MVK
24	Localization of nervonic acid beta-oxidation in human and rodent peroxisomes: impaired oxidation in Zellweger syndrome and X-linked adrenoleukodystrophy. (9799802)	Sandhir R.... Singh I.	1998	ABCD1
25	Human PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group I. (9539740)	Tamura S.... Fujiki Y.	1998	PEX1
26	A novel mutation, R125X in peroxisome assembly factor-1 responsible for Zellweger syndrome. (9452066)	Shimozawa N.... Kondo N.	1998	PEX2
27	Mutation in PEX16 is causal in the peroxisome-deficient Zellweger syndrome of complementation group D. (9837814)	Honsho M.... Fujiki Y.	1998	PEX16
28	Disruption of a PEX1-PEX6 interaction is the most common cause of the neurologic disorders Zellweger syndrome, neonatal adrenoleukodystrophy, and infantile Refsum disease. (9671729)	Geisbrecht B.V.... Gould S.J.	1998	PEX1, PEX6
29	Molecular basis of Zellweger syndrome, beta-ketothiolase deficiency and mucopolysaccharidoses. (9183994)	Orii T.	1997	GALNS, PEX6, PEX2
30	The human peroxisomal multifunctional protein involved in bile acid synthesis: activity measurement, deficiency in Zellweger syndrome and chromosome mapping. (9197465)	Novikov D.... Van Veldhoven P.P.	1997	HSD17B4
31	Phytanoyl-CoA hydroxylase is present in human liver, located in peroxisomes, and deficient in Zellweger syndrome: direct, unequivocal evidence for the new, revised pathway of phytanic acid alpha-oxidation in humans. (8954107)	Jansen G.A.... Wanders R.J.	1996	PHYH
32	Cholesterol biosynthesis in Zellweger syndrome: normal activity of mevalonate kinase, mevalonate-5'-pyrophosphate decarboxylase and IPP-isomerase in patients' fibroblasts but deficient mevalonate kinase activity in liver. (8739963)	Wanders R.J.... Romeijn G.J.	1996	MVK, IDI1
33	Molecular characterization of the human peroxisomal branched-chain acyl-CoA oxidase: cDNA cloning, chromosomal assignment, tissue distribution, and evidence for the absence of the protein in Zellweger's syndrome. (8943006)	Baumgart E.... Van Veldhoven P.P.	1996	ACOX1, ACOX2
34	Zellweger syndrome and associated phenotypes. (8933342)	FitzPatrick D.R.	1996	GNPAT
35	Effect of hypoxia-reoxygenation on peroxisomal functions in cultured human skin fibroblasts from control and Zellweger syndrome patients. (7889146)	Kremser K.... Singh I.	1995	GNPAT
36	A nonmammalian homolog of the PAF1 gene (Zellweger syndrome) discovered as a gene involved in caryogamy in the fungus Podospora anserina. (7600573)	Berteaux-Lecellier V.... Simonet J.M.	1995	PEX2
37	The pas8 mutant of Pichia pastoris exhibits the peroxisomal protein import deficiencies of Zellweger syndrome cells--the PAS8 protein binds to the COOH-terminal tripeptide peroxisomal targeting signal, and is a member of the TPR protein family. (8098333)	McCollum D.... Subramani S.	1993	PEX5
38	Peroxisome assembly mutations in humans: structural heterogeneity in Zellweger syndrome. (1560037)	Santos M.J.... Lazarow P.B.	1992	CAT
39	A human gene responsible for Zellweger syndrome that affects peroxisome assembly. (1546315)	Shimozawa N.... Fujiki Y.	1992	PEX2
40	Zellweger syndrome in a preterm, small for gestationa l age infant. (1583879)	Samsom J.F.... Wanders R.J.	1992	GNPAT
41	Mutations in the 70K peroxisomal membrane protein gene in Zellweger syndrome. (1301993)	Gaertner J.... Valle D.	1992	ABCD3
42	Low-density particles (W-particles) containing catalase in Zellweger syndrome and normal fibroblasts. (1946426)	Aikawa J.... Chen G.L.	1991	CAT
43	Identification of pristanoyl-CoA oxidase and phytanic acid decarboxylation in peroxisomes and mitochondria from human liver: implications for Zellweger syndrome. (1770789)	Wanders R.J.... ten Brink H.J.	1991	ACOX3
44	Acyl-CoA oxidase, peroxisomal thiolase and dihydroxyacetone phosphate acyltransferase: aberrant subcellular localization in Zellweger syndrome. (1679469)	Van Roermund C.W.... Wanders R.J.	1991	ACOX1, GNPAT
45	Aberrant subcellular localization of peroxisomal 3-ketoacyl-CoA thiolase in the Zellweger syndrome and rhizomelic chondrodysplasia punctata. (2181395)	Balfe A.... Watkins P.A.	1990	HADHB
46	Identification of pristanoyl-CoA oxidase activity in human liver and its deficiency in the Zellweger syndrome. (2241949)	Wanders R.J.... Jakobs C.	1990	ACOX3
47	Gene assignment of Zellweger syndrome to 7q11.23: report of the second case associated with a pericentric inversion of chromosome 7. (2606480)	Naritomi K.... Hirayama K.	1989	PEX1
48	Genetic heterogeneity in the cerebrohepatorenal (Zellweger) syndrome and other inherited disorders with a generalized impairment of peroxisomal functions. A study using complementation analysis. (2454948)	Brul S.... Tager J.M.	1988	PEX2
49	Properties of the enzymes catalyzing the biosynthesis of lysophosphatidate and its ether analog in cultured fibroblasts from Zellweger syndrome patients and normal controls. (3646870)	Webber K.O.... Hajra A.K.	1987	AGPS
50	Alkyl dihydroxyacetone phosphate synthase in human fibroblasts and its deficiency in Zellweger syndrome. (4031664)	Schrakamp G.... van den Bosch H.	1985	AGPS

Genes related to zellweger syndrome according to GeneCards:

(show top 50)    (show all 54)

id	Symbol	Description	Score	GeneCards Section Context
1	PEX1	peroxisomal biogenesis factor 1	11.1	Publications, Aliases & Descriptions, Summaries
2	PEX2	peroxisomal biogenesis factor 2	11.1	Publications, Aliases & Descriptions, Orthologs, Summaries
3	ABCD3	ATP-binding cassette, sub-family D (ALD), member 3	11.1	UniProt Related, Summaries, Aliases & Descriptions, Publications
4	PEX16	peroxisomal biogenesis factor 16	11.1	Publications, Summaries
5	PEX3	peroxisomal biogenesis factor 3	11.1	Orthologs, Publications, Summaries
6	PEX12	peroxisomal biogenesis factor 12	11.1	Summaries, Publications
7	PEX10	peroxisomal biogenesis factor 10	11.1	Summaries, Publications
8	PEX26	peroxisomal biogenesis factor 26	11.1	Summaries, Publications
9	PEX19	peroxisomal biogenesis factor 19	11.1	Publications, Summaries, Orthologs
10	PEX5	peroxisomal biogenesis factor 5	11.1	Orthologs, Publications, Summaries
11	PEX13	peroxisomal biogenesis factor 13	11.1	Orthologs, Publications, Summaries
12	ACAA1	acetyl-CoA acyltransferase 1	11.1	Summaries
13	PEX14	peroxisomal biogenesis factor 14	11.1	Publications, Summaries
14	AGPS	alkylglycerone phosphate synthase	11.1	Summaries, Publications
15	EHHADH	enoyl-CoA, hydratase/3-hydroxyacyl CoA dehydrogenase	11.1	Summaries, UniProt Related
16	SCP2	sterol carrier protein 2	11.1	Summaries
17	IDI1	isopentenyl-diphosphate delta isomerase 1	11.1	Summaries, Publications
18	PHYH	phytanoyl-CoA 2-hydroxylase	11.1	Summaries, Publications
19	ACOX2	acyl-CoA oxidase 2, branched chain	11.1	Summaries, Publications
20	ABCD2	ATP-binding cassette, sub-family D (ALD), member 2	11.1	Summaries
21	PEX6	peroxisomal biogenesis factor 6	11.0	Publications
22	GNPAT	glyceronephosphate O-acyltransferase	11.0	Publications
23	PMVK	phosphomevalonate kinase	11.0
24	PIPOX	pipecolic acid oxidase	11.0
25	MVK	mevalonate kinase	11.0	Publications
26	ACOX3	acyl-CoA oxidase 3, pristanoyl	11.0	Publications
27	ACOX1	acyl-CoA oxidase 1, palmitoyl	11.0	Publications
28	SLC25A17	solute carrier family 25 (mitochondrial carrier; peroxisomal membrane protein, 34kDa), member 17	11.0	Publications
29	HSD17B4	hydroxysteroid (17-beta) dehydrogenase 4	11.0	Publications
30	DAO	D-amino-acid oxidase	11.0	Publications
31	HADHB	hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit	11.0	Publications
32	NES	nestin	11.0	Publications
33	DCX	doublecortin	11.0	Publications
34	AMACR	alpha-methylacyl-CoA racemase	11.0
35	CAT	catalase	11.0	Publications
36	PHEX	phosphate regulating endopeptidase homolog, X-linked	11.0
37	PEX11A	peroxisomal biogenesis factor 11 alpha	11.0
38	PGAM5	phosphoglycerate mutase family member 5	11.0	Disorders
39	DBI	diazepam binding inhibitor (GABA receptor modulator, acyl-CoA binding protein)	11.0	Publications
40	PEX11B	peroxisomal biogenesis factor 11 beta	11.0
41	HACL1	2-hydroxyacyl-CoA lyase 1	11.0	Publications
42	ABCG4	ATP-binding cassette, sub-family G (WHITE), member 4	11.0
43	ACAA2	acetyl-CoA acyltransferase 2	11.0
44	CCDC62	coiled-coil domain containing 62	11.0	Disorders
45	LAMP2	lysosomal-associated membrane protein 2	11.0	Publications
46	ARSH	arylsulfatase family, member H	11.0	Publications
47	ABCD1	ATP-binding cassette, sub-family D (ALD), member 1	11.0	Publications
48	NR1I3	nuclear receptor subfamily 1, group I, member 3	11.0	Publications
49	PEX7	peroxisomal biogenesis factor 7	11.0
50	GALNS	galactosamine (N-acetyl)-6-sulfate sulfatase	11.0	Publications

Pathways related to zellweger syndrome according to GeneDecks:

(show all 13)

id	Pathway	Score	Top Affiliating Genes
1	Biosynthesis of unsaturated fatty acids20	10.1	ACAA1, ACOX1, ACOX3
2	ABCA transporters in lipid homeostasis38	10.1	PEX3, PEX19, ABCG4, ABCD3, ABCD2
3	Primary bile acid biosynthesis20	10.1	SCP2, AMACR, HSD17B4, ACOX2
4	Terpenoid backbone biosynthesis20	10.0	PMVK, IDI1, MVK
5	Activation of Gene Expression by SREBP (SREBF)38	9.8	MVK, IDI1, PMVK
6	ABC transporters20	9.7	ABCD1, ABCD2, ABCD3, ABCG4
7	Fatty acid elongation in mitochondria20	9.6	HADHB, HADH, ACAA2
8	Valine, leucine and isoleucine degradation20	9.5	HADHB, HADH, EHHADH, ACAA2, ACAA1
9	PPAR signaling pathway20	9.3	EHHADH, DBI, ACOX3, ACOX1, ACOX2, ACAA1
10	Fatty acid metabolism20	9.2	HADHB, HADH, EHHADH, ACOX3, ACOX1, ACAA2
11	Metabolism of lipids and lipoproteins38	7.4	HADH, ARSH, SCP2, AMACR, GNPAT, HACL1
12	Metabolic pathways20	7.4	ACOX1, ACOX3, AGPS, PMVK, EHHADH, HADH
13	Peroxisome20	6.0	HACL1, EHHADH, PEX7, PEX6, PEX5, PEX3

Compounds related to zellweger syndrome according to GeneDecks:

(show all 47)

id	Compound	Score	Top Affiliating Genes
1	3-oxoacyl-coa32	10.5	EHHADH, ACAA1, GNPAT, SCP2
2	(2S,6R,10R)-Trimethyl-hendecanoyl-CoA18	10.5	ACOX3, ACOX1
3	3-methylcrotonyl-coa32 18	11.4	HADHB, EHHADH, ACAA1
4	mevalonate pyrophosphate32	10.4	MVK, PMVK
5	isopentenyl diphosphate32	10.4	PMVK, IDI1, MVK
6	benzoyl-coa32 18	11.4	HADHB, ACAA1
7	2-hydroxyphytanoyl-coa32 18	11.4	HACL1, PHYH
8	tripeptide32	10.3	ACOX2, ACOX1, ACOX3, PEX5
9	acetoacetyl coa32	10.3	HADHB, PMVK, ACAA2
10	salicylhydroxamic acid32	10.3	ACOX1, CAT
11	pipecolic acid32 18	11.3	DAO, PIPOX
12	lignoceric acid32	10.2	HADHB, ABCD1, GNPAT
13	trimetazidine32	10.1	CAT, HADHB
14	(S)-3-hydroxypalmitoleoyl-CoA18	10.1	HADH, EHHADH, HSD17B4
15	(S)-Hydroxyhexanoyl-CoA18	10.1	HSD17B4, EHHADH, HADH
16	(S)-Hydroxyoctanoyl-CoA18	10.1	HSD17B4, EHHADH, HADH
17	(S)-Hydroxydecanoyl-CoA18	10.1	HADH, EHHADH, HSD17B4
18	(S)-3-Hydroxydodecanoyl-CoA18	10.1	HSD17B4, EHHADH, HADH
19	(S)-3-Hydroxytetradecanoyl-CoA18	10.1	HSD17B4, EHHADH, HADH
20	2-methyl-3-hydroxybutyryl-coa32 18	11.1	HADH, EHHADH, HSD17B4
21	(S)-3-Hydroxyhexadecanoyl-CoA18	10.0	HSD17B4, EHHADH, HADH
22	(S)-Methylmalonic acid semialdehyde18	10.0	HADH, EHHADH, HSD17B4
23	clofibric acid32	10.0	ACOX1, PPARA, CAT
24	Coenzyme A9 18 9	11.9	HADHB, DBI, ACAA2, ACAA1, GNPAT
25	(3S)-3-Hydroxyadipyl-CoA18	9.9	HADHB, HADH, EHHADH, HSD17B4
26	clofibrate32 9 9	11.9	HSD17B4, PPARA, EHHADH
27	phosphomevalonate32	9.9	MVK, PMVK
28	ubiquinone32	9.9	MVK, CAT, ACOX1
29	ciprofibrate32	9.9	ACOX1, PPARA, CAT
30	n-acetylgalactosamine 6-sulfate32	9.9	ARSH, GALNS
31	pristanic acid32 18	10.6	EHHADH, ACOX3, ACOX2, PPARA, GNPAT, AMACR
32	phytanic acid32	9.2	HACL1, ACOX3, ACAA1, PHYH, PPARA, CAT
33	carnitine32	9.1	HADHB, HADH, ACOX1, PPARA
34	2-methylbutyryl-coa32 18	10.1	HADHB, DBI, ACOX3, ACOX1, ACAA2, ACOX2
35	octanoyl-coa32 18	10.1	HADHB, DBI, ACOX3, ACOX1, ACAA2, ACOX2
36	stearoyl-coa32 18	10.1	HADHB, DBI, ACOX3, ACOX1, ACAA2, ACOX2
37	(2E)-Octenoyl-CoA18	9.0	EHHADH, DBI, ACOX3, ACOX1, ACAA2, ACOX2
38	(2E)-Tetradecenoyl-CoA18	9.0	GNPAT, PPARA, HADHB, EHHADH, DBI, ACOX3
39	(2E)-Dodecenoyl-CoA18	9.0	HADHB, GNPAT, EHHADH, DBI, ACOX3, ACOX1
40	(2E)-Decenoyl-CoA18	9.0	DBI, ACOX3, ACOX1, ACAA2, ACOX2, ACAA1
41	(2E)-Hexadecenoyl-CoA18	9.0	HADHB, EHHADH, DBI, ACOX3, ACOX1, ACAA2
42	acetyl-coa32 18	10.0	HADHB, DBI, ACOX3, ACOX1, ACAA2, ACOX2
43	sterol32	8.4	ABCD2, NR1I3, PPARA, HSD17B4, AMACR, MVK
44	cholesterol32 9 18 9	10.7	ABCD1, ABCD2, ABCG4, DBI, PMVK, EHHADH
45	acyl-coa32	7.5	SCP2, SLC25A17, DBI, ACOX3, ACOX1, ACOX2
46	lipid32	7.5	ABCD3, ACOX1, DBI, PEX1, PEX2, PEX5
47	fatty acid32	7.3	HADHB, HADH, EHHADH, PEX7, DBI, AGPS

Cellular components related to zellweger syndrome according to GeneDecks:

id	Name	GO ID	Score	Top Affiliating Genes
1	intracellular membrane-bounded organelle	GO:043231	9.6	PEX3, PEX1, AGPS, ACAA1, ABCD3, ABCD2
2	integral to peroxisomal membrane	GO:005779	9.0	PEX10, PEX11A, PEX11B, PEX12, PEX13, PEX16
3	peroxisomal matrix	GO:005782	8.2	SCP2, AMACR, GNPAT, HSD17B4, CAT, DAO
4	peroxisomal membrane	GO:005778	7.8	ACOX1, PEX2, PEX3, PEX5, PEX6, PEX19
5	mitochondrion	GO:005739	7.4	DBI, SLC25A17, PEX11B, PEX5, EHHADH, HADH
6	peroxisome	GO:005777	5.6	PIPOX, ABCD3, ACAA1, ACOX2, ACOX1, ACOX3

Biological processes related to zellweger syndrome according to GeneDecks:

(show all 25)

id	Name	GO ID	Score	Top Affiliating Genes
1	protein import into peroxisome membrane	GO:045046	10.4	PEX5, PEX3, PEX26, PEX19, PEX16
2	protein import into peroxisome matrix, translocation	GO:016561	10.4	PEX6, PEX5, PEX14
3	negative regulation of protein homotetramerization	GO:1901094	10.4	PEX5, PEX14
4	peroxisome membrane biogenesis	GO:016557	10.3	PEX3, PEX19, PEX16, PEX11A
5	microtubule-based peroxisome localization	GO:060152	10.3	PEX13, PEX1
6	protein targeting to peroxisome	GO:006625	10.3	PEX6, PEX5, PEX19, PEX16, PEX12, PEX1
7	very long-chain fatty acid metabolic process	GO:000038	10.3	ABCD2, ACAA1, ACOX1, PEX5
8	protein import into peroxisome matrix, docking	GO:016560	10.2	PEX5, PEX13
9	fatty acid alpha-oxidation	GO:001561	10.2	HACL1, PEX13, SLC25A17, PHYH
10	bile acid biosynthetic process	GO:006699	10.2	SCP2, AMACR, HSD17B4, ACOX2, PEX2
11	very long-chain fatty acid catabolic process	GO:042760	10.1	ABCD3, ABCD2, ABCD1
12	bile acid metabolic process	GO:008206	10.1	ACOX2, ACAA1, HSD17B4, AMACR, SCP2
13	regulation of peroxisome size	GO:044375	10.0	PEX11A, PEX11B
14	ether lipid biosynthetic process	GO:008611	10.0	PEX7, AGPS, GNPAT
15	alpha-linolenic acid metabolic process	GO:036109	10.0	ACOX1, ACAA1, ABCD1, HSD17B4, SCP2
16	cholesterol biosynthetic process	GO:006695	10.0	PMVK, ACAA2, IDI1, MVK
17	unsaturated fatty acid metabolic process	GO:033559	10.0	ACOX1, ACAA1, ABCD1, HSD17B4, SCP2
18	isopentenyl diphosphate biosynthetic process, mevalonate pathway	GO:019287	9.9	MVK, PMVK
19	protein import into peroxisome matrix	GO:016558	9.8	PEX7, PEX5, PEX26, PEX2, PEX1, PEX10
20	fatty acid beta-oxidation using acyl-CoA oxidase	GO:033540	9.6	SCP2, AMACR, HSD17B4, ABCD1, ACAA1, ACOX2
21	neuron migration	GO:001764	9.0	PEX7, PEX5, PEX2, PEX13, DCX, DCLK1
22	peroxisome organization	GO:007031	8.9	PEX1, PEX7, PEX6, PEX3, PEX10, PEX11A
23	fatty acid beta-oxidation	GO:006635	8.3	HSD17B4, ABCD1, ABCD2, ABCD3, ACAA1, ACAA2
24	cellular lipid metabolic process	GO:044255	7.5	ACOX1, ACOX3, AGPS, SLC25A17, PEX11A, HACL1
25	small molecule metabolic process	GO:044281	6.0	SCP2, ACAA1, ACOX2, ACOX1, ACOX3, AGPS

Molecular functions related to zellweger syndrome according to GeneDecks:

(show all 16)

id	Name	GO ID	Score	Top Affiliating Genes
1	pristanoyl-CoA oxidase activity	GO:016402	10.4	ACOX3, ACOX2
2	acyl-CoA dehydrogenase activity	GO:003995	10.3	ACOX3, ACOX1, ACOX2
3	long-chain-enoyl-CoA hydratase activity	GO:016508	10.3	HADHB, HSD17B4
4	acetyl-CoA C-acyltransferase activity	GO:003988	10.2	ACAA1, ACAA2, HADHB
5	acyl-CoA oxidase activity	GO:003997	10.2	ACOX2, ACOX1
6	sterol binding	GO:032934	10.2	SCP2, HSD17B4
7	protein C-terminus binding	GO:008022	10.2	PEX6, PEX5, PEX26, PEX16, PEX12, PEX10
8	flavin adenine dinucleotide binding	GO:050660	10.1	AGPS, ACOX3, ACOX1, ACOX2
9	protein N-terminus binding	GO:047485	10.0	PEX5, PEX19, PEX14, ACOX1
10	enoyl-CoA hydratase activity	GO:004300	9.9	EHHADH, HADHB
11	3-hydroxyacyl-CoA dehydrogenase activity	GO:003857	9.8	HADHB, HADH, EHHADH, HSD17B4
12	peroxisome targeting sequence binding	GO:000268	9.8	PEX5, PEX5L
13	ATPase activity	GO:016887	9.8	PEX6, ABCG4, ABCD3, ABCD2, ABCD1
14	receptor binding	GO:005102	8.9	PIPOX, HACL1, EHHADH, PEX14, ACOX3, ACOX1
15	protein homodimerization activity	GO:042803	8.8	PEX7, PEX11B, PEX11A, ABCG4, ABCD3, ABCD2
16	protein binding	GO:005515	7.2	PEX12, PEX13, PEX14, PEX16, PEX19, PEX2