Peroxisomal protein import

Pathway network for the Peroxisomal protein import SuperPath

Sources:
  • Reactome
  • WikiPathways
  • PubChem

Pathways in the Peroxisomal protein import SuperPath

#NameSourceGenes
1Peroxisomal protein importReactome
2Protein localizationReactome
3Mitochondrial protein importReactome
4Peroxisomal lipid metabolismReactome
5Class I peroxisomal membrane protein importReactome
6Beta-oxidation of very long chain fatty acidsReactome
7Beta-oxidation of pristanoyl-CoAReactome
8TYSND1 cleaves peroxisomal proteinsReactome
9Alpha-oxidation of phytanateReactome
10Peroxisomal beta-oxidation of tetracosanoyl-CoAWikiPathways
11phytol degradationPubChem

Gene overlap in member pathways for Peroxisomal protein import SuperPath

Ordered by rank within the SuperPath, via the multiplicity of each gene in the constituent pathways

Disorders associated with Peroxisomal protein import SuperPath

according to GeneCards Suite gene sharing
#DisorderTypeGenesScore
1Neonatal adrenoleukodystrophyEnrichmentPEX11B, PEX12, PEX13, PEX14, PEX16, PEX19, PEX2, PEX26, PEX311.32
2Peroxisome biogenesis disorder 1bEnrichmentPEX11B, PEX12, PEX13, PEX14, PEX16, PEX19, PEX2, PEX26, PEX310.94
3Zellweger syndromeEnrichmentPEX11B, PEX12, PEX13, PEX14, PEX16, PEX19, PEX2, PEX26, PEX310.94
4Zellweger spectrum disorderEnrichmentPEX1, PEX10, PEX19, PEX2, PEX5, PEX69.94
5Rhizomelic chondrodysplasia punctataEnrichmentAGPS, GNPAT, PEX5, PEX79.35
6Peroxisome biogenesis disorder 1aEnrichmentPEX1, PEX3, PEX5, PEX66.54
7D-bifunctional protein deficiencyEnrichmentEHHADH, HSD17B45.75
8Peroxisome biogenesis disorder 2bEnrichmentPEX5, PEX64.67
9Refsum disease, classicEnrichmentPEX7, PHYH4.20
10Heimler syndrome 1EnrichmentPEX1, PEX63.90
11Sjogren-larsson syndromeEnrichmentALDH3A23.53
12Peroxisomal acyl-coa oxidase deficiencyEnrichmentACOX13.53
13Mitchell syndromeEnrichmentACOX13.53
14Leukoencephalopathy with dystonia and motor neuropathyEnrichmentSCP23.53
15Rhizomelic chondrodysplasia punctata, type 3EnrichmentAGPS3.29
16Carnitine acetyltransferase deficiencyEnrichmentCRAT3.18
17Bile acid synthesis defect, congenital, 6EnrichmentACOX23.18
18Neurodegeneration with brain iron accumulation 8EnrichmentCRAT3.18
19Hypoadrenocorticism, familialEnrichmentABCD13.09
20Fanconi renotubular syndrome 3EnrichmentEHHADH3.09
21EncephalitisEnrichmentABCD13.09
22X-linked cerebral adrenoleukodystrophyEnrichmentABCD13.09
23AdrenomyeloneuropathyEnrichmentABCD13.09
24Chronic primary adrenal insufficiencyEnrichmentABCD13.09
25Isolated complex iii deficiencyEnrichmentBCS1L, CYC12.94
26Bile acid synthesis defect, congenital, 4EnrichmentAMACR2.88
27Alpha-methylacyl-coa racemase deficiencyEnrichmentAMACR2.88
28Peroxisome biogenesis disorder 3bEnrichmentPEX122.83
29Peroxisome biogenesis disorder 10aEnrichmentPEX32.83
30Peroxisome biogenesis disorder 3aEnrichmentPEX122.83
31Peroxisome biogenesis disorder 8aEnrichmentPEX162.83
32Peroxisome biogenesis disorder 12aEnrichmentPEX192.83
33Peroxisome biogenesis disorder 14bEnrichmentPEX11B2.83
34Retinal dystrophy with leukodystrophyEnrichmentACBD52.83
35Peroxisome biogenesis disorder 10bEnrichmentPEX32.83
36Peroxisome biogenesis disorder 8bEnrichmentPEX162.83
37Bile acid synthesis defect, congenital, 5EnrichmentABCD32.83
38Hyperekplexia 4EnrichmentATAD12.83
39Autosomal recessive ataxia due to pex16 deficiencyEnrichmentPEX162.83
40Autosomal recessive ataxia due to pex2 deficiencyEnrichmentPEX22.83
41Spondyloepimetaphyseal dysplasia, x-linkedEnrichmentABCD12.79
42AdrenoleukodystrophyEnrichmentABCD12.79
43CaddsEnrichmentABCD12.79
44Perrault syndromeEnrichmentHSD17B42.63
45Ehlers-danlos syndrome, kyphoscoliotic type, 1EnrichmentABCD12.61
46Plod1-related kyphoscoliotic ehlers-danlos syndromeEnrichmentABCD12.61
47Perrault syndrome 2EnrichmentHSD17B42.58
48Early-onset autosomal dominant alzheimer diseaseEnrichmentAPP, TOMM402.54
49Charcot-marie-tooth disease, demyelinating, type 4aEnrichmentGDAP12.53
50Charcot-marie-tooth disease, axonal, with vocal cord paresis, autosomal recessiveEnrichmentGDAP12.53
51Charcot-marie-tooth disease, recessive intermediate aEnrichmentGDAP12.53
52Peroxisome biogenesis disorder 11aEnrichmentPEX132.53
53Peroxisome biogenesis disorder 13aEnrichmentPEX142.53
54Peroxisome biogenesis disorder 11bEnrichmentPEX132.53
55Charcot-marie-tooth disease, axonal, type 2hEnrichmentGDAP12.53
56Peroxisome biogenesis disorder 7aEnrichmentPEX262.53
57Axonal neuropathyEnrichmentGDAP12.53
58Peroxisome biogenesis disorder 7bEnrichmentPEX262.53
59Charcot-marie-tooth disease type 4aEnrichmentGDAP12.53
60Congenital nervous system abnormalityEnrichmentABCD1, HSD17B42.50
61Nervous system diseaseEnrichmentABCD1, HSD17B42.50
62Malonyl-coa decarboxylase deficiencyEnrichmentMLYCD2.49
63Primary fanconi renotubular syndromeEnrichmentEHHADH2.49
64Orofacial cleft 1EnrichmentPHYH2.40
65Peroxisome biogenesis disorder 5bEnrichmentPEX22.35
66Charcot-marie-tooth disease, axonal, type 2kEnrichmentGDAP12.35
67Peroxisome biogenesis disorder 5aEnrichmentPEX22.35
68Rhizomelic chondrodysplasia punctata, type 2EnrichmentGNPAT2.33
69Peroxisome biogenesis disorder 2aEnrichmentPEX52.33
70Intellectual developmental disorder, x-linked 99EnrichmentUSP9X2.33
71Rhizomelic chondrodysplasia punctata, type 1EnrichmentPEX72.33
723-hydroxy-3-methylglutaryl-coa lyase deficiencyEnrichmentHMGCL2.33
73Hyperoxaluria, primary, type iEnrichmentAGXT2.33
74Peroxisome biogenesis disorder 4aEnrichmentPEX62.33
75Intellectual developmental disorder, x-linked 99, syndromic, female-restrictedEnrichmentUSP9X2.33
76AcatalasemiaEnrichmentCAT2.33
77Peroxisome biogenesis disorder 9bEnrichmentPEX72.33
78Peroxisome biogenesis disorder 6aEnrichmentPEX102.33
79Peroxisome biogenesis disorder 6bEnrichmentPEX102.33
80Metaphyseal enchondromatosis with d-2-hydroxyglutaric aciduriaEnrichmentIDH12.33
81Heimler syndrome 2EnrichmentPEX62.33
82Hypercholanemia, familial 3EnrichmentBAAT2.33
83Rhizomelic chondrodysplasia punctata, type 5EnrichmentPEX52.33
84Female-restricted syndromic x-linked intellectual disability 99EnrichmentUSP9X2.33
85Peroxisomal diseaseEnrichmentPEX12.33
86Autosomal recessive ataxia due to pex10 deficiencyEnrichmentPEX102.33
87D-lactic aciduria with goutEnrichmentLDHD2.33
88Retinitis pigmentosa 99EnrichmentIDH3G2.33
89Mohr-tranebjaerg syndromeEnrichmentTIMM8A2.33
90Developmental and epileptic encephalopathy 39 with leukodystrophyEnrichmentSLC25A122.33
913-methylglutaconic aciduria, type vEnrichmentDNAJC192.33
92Mitochondrial complex iii deficiency, nuclear type 6EnrichmentCYC12.33
93Even-plus syndromeEnrichmentHSPA92.33
94Anemia, sideroblastic, 4EnrichmentHSPA92.33
95Spinal muscular atrophy, jokela typeEnrichmentCHCHD102.33
96Bjornstad syndromeEnrichmentBCS1L2.33
97Myopathy, isolated mitochondrial, autosomal dominantEnrichmentCHCHD102.33
98Mandibuloacral dysplasia progeroid syndromeEnrichmentMTX22.33
99Ornithine transcarbamylase deficiency, hyperammonemia due toEnrichmentOTC2.33
100Mitochondrial complex v deficiency, nuclear type 4bEnrichmentATP5F1A2.33
101Gracile syndromeEnrichmentBCS1L2.33
102Mitochondrial complex iv deficiency, nuclear type 13EnrichmentCOA62.33
103Spastic paraplegia 13, autosomal dominantEnrichmentHSPD12.33
104Combined oxidative phosphorylation deficiency 22EnrichmentATP5F1A2.33
105Parkinson disease 22, autosomal dominantEnrichmentCHCHD22.33
106Spinocerebellar ataxia, autosomal recessive 30EnrichmentPITRM12.33
1073-methylglutaconic aciduria, type ixEnrichmentTIMM502.33
108Mitochondrial complex i deficiency, nuclear type 32EnrichmentNDUFB82.33
109Mitochondrial complex v deficiency, nuclear type 4aEnrichmentATP5F1A2.33
110Combined oxidative phosphorylation deficiency 43EnrichmentTIMM222.33
111Developmental and epileptic encephalopathy 39EnrichmentSLC25A122.33
112Garg-mishra progeroid syndromeEnrichmentTOMM72.33
113Anemia, sideroblastic, 5EnrichmentHSCB2.33
114Hypermetabolism due to uncoupled mitochondrial oxidative phosphorylation 2EnrichmentATP5F1B2.33
115Lower motor neuron syndrome with late-adult onsetEnrichmentCHCHD102.33
116Renal tubulopathy-encephalopathy-liver failure syndromeEnrichmentBCS1L2.33
117Disorder of ornithine metabolismEnrichmentOTC2.33
118Multiple system atrophy, parkinsonian typeEnrichmentCOQ22.33
119Pigmentation anomaly of the skinEnrichmentMTX22.33
120Citrullinemia type iiEnrichmentSLC25A132.33
121Perrault syndrome 1EnrichmentHSD17B42.33
122Cerebral palsyEnrichmentALDH3A22.24
123MyocarditisEnrichmentABCD12.19
124Neurodegeneration with brain iron accumulationEnrichmentCRAT2.18
125HyperekplexiaEnrichmentATAD12.13
126PolyneuropathyEnrichmentGDAP12.13
127Sensory peripheral neuropathyEnrichmentGDAP12.13
128Peroxisome biogenesis disorder 4bEnrichmentPEX62.03
129Mitochondrial dna depletion syndrome 15EnrichmentMPV172.03
130Buratti-harel syndromeEnrichmentLONP22.03
131Primary hyperoxaluriaEnrichmentAGXT2.03
132Submucosal cleft palateEnrichmentUBB2.03
133Cleft hard palateEnrichmentUBB2.03
134Multiple system atrophy 1EnrichmentCOQ22.03
135Citrullinemia, classicEnrichmentSLC25A132.03
136Hydrocephalus, normal-pressure, 1EnrichmentPMPCA2.03
137Leukodystrophy, hypomyelinating, 4EnrichmentHSPD12.03
138Porphyria, acute intermittentEnrichmentACO22.03
139Citrin deficiency, neonatal or infantile onsetEnrichmentSLC25A132.03
140Myopathy, mitochondrial progressive, with congenital cataract and developmental delayEnrichmentGFER2.03
141Spondylometaphyseal dysplasia, megarbane-dagher-melki typeEnrichmentPAM162.03
142Frontotemporal dementia and/or amyotrophic lateral sclerosis 2EnrichmentCHCHD102.03
143Agammaglobulinemia, x-linkedEnrichmentTIMM8A2.03
144Multiple mitochondrial dysfunctions syndrome 6EnrichmentPMPCB2.03
145Isolated mitochondrial myopathyEnrichmentCHCHD102.03
146Spondylometaphyseal dysplasia megarbane-dagher-melike typeEnrichmentPAM162.03
147Acute porphyriaEnrichmentACO22.03
148Autosomal recessive sideroblastic anemiaEnrichmentHSPA92.03
149CitrullinemiaEnrichmentSLC25A132.03
150Normal pressure hydrocephalusEnrichmentPMPCA2.03
151Multiple system atrophyEnrichmentCOQ22.03
152Multiple system atrophy, cerebellar typeEnrichmentCOQ22.03
153Oculopharyngodistal myopathy 1EnrichmentABCD31.99
154Fatal familial insomniaEnrichmentPRNP1.92
155Gerstmann-straussler diseaseEnrichmentPRNP1.92
156KuruEnrichmentPRNP1.92
157Cardiomyopathy, dilated, 2hEnrichmentGET31.92
158Congenital disorder of glycosylation, type iizEnrichmentCAMLG1.92
159Spongiform encephalopathy with neuropsychiatric featuresEnrichmentPRNP1.92
160Huntington disease-like 1EnrichmentPRNP1.92
161Arthrogryposis and ectodermal dysplasiaEnrichmentOTOF1.92
162Cerebral amyloid angiopathy, app-relatedEnrichmentAPP1.92
163Congenital disorder of glycosylation, type iiaaEnrichmentSTX51.92
164Familial alzheimer-like prion diseaseEnrichmentPRNP1.92
165Neurodevelopmental disorder with hypotonia and autistic features with or without hyperkinetic movementsEnrichmentVAMP21.92
166Heme oxygenase 1 deficiencyEnrichmentHMOX11.92
167Prion diseaseEnrichmentPRNP1.92
168Congenital disorder of glycosylation, type iiyEnrichmentGET41.92
169Prp systemic amyloidosisEnrichmentPRNP1.92
170Inherited human prion diseaseEnrichmentPRNP1.92
171Inherited creutzfeldt-jakob diseaseEnrichmentPRNP1.92
172Uvula, bifidEnrichmentUBB1.86
173Cleft soft palateEnrichmentUBB1.86
174Long-chain 3-hydroxyacyl-coa dehydrogenase deficiencyEnrichmentHMGCL1.86
175Brown-vialetto-van laere syndrome 2EnrichmentPEX61.86
176EnchondromatosisEnrichmentIDH11.86
177Mpv17-related mitochondrial dna maintenance defectEnrichmentMPV171.86
178Hyperpigmentation of the skinEnrichmentUSP9X1.86
179Mitochondrial complex iii deficiency, nuclear type 1EnrichmentBCS1L1.86
180Mitochondrial complex iv deficiency, nuclear type 5EnrichmentATP5F1A1.86
181Friedreich ataxiaEnrichmentFXN1.86
182Spinocerebellar ataxia, autosomal recessive 2EnrichmentPMPCA1.86
183Citrin deficiency, adolescent or adult onsetEnrichmentSLC25A131.86
184Barth syndromeEnrichmentTAFAZZIN1.86
185Mitochondrial dna depletion syndrome 7EnrichmentPITRM11.86
186Coenzyme q10 deficiency, primary, 1EnrichmentCOQ21.86
187Infantile cerebellar-retinal degenerationEnrichmentACO21.86
188Optic atrophy 9EnrichmentACO21.86
189Optic atrophy plus syndromeEnrichmentACO2, PEX1, PMPCA1.81
190Mitochondrial dna depletion syndrome 6EnrichmentMPV171.73
191Charcot-marie-tooth disease, axonal, type 2eeEnrichmentMPV171.73
192Autosomal recessive isolated optic atrophyEnrichmentACO21.73
193Enchondromatosis, multiple, ollier typeEnrichmentIDH11.64
194Paroxysmal extreme pain disorderEnrichmentIDH11.64
195Cox deficiency, infantile mitochondrial myopathyEnrichmentCOA61.64
196Rare genetic deafnessEnrichmentHSD17B41.64
197Hirschsprung disease 1EnrichmentABCD11.63
198Creatine phosphokinase, elevated serumEnrichmentGDAP11.63
199Isolated elevated serum creatine phosphokinase levelsEnrichmentGDAP11.63
200Cerebral amyloid angiopathy, cst3-relatedEnrichmentAPP1.62
201Methemoglobinemia and ambiguous genitaliaEnrichmentCYB5A1.62
202Myopathy, x-linked, with postural muscle atrophyEnrichmentEMD1.62
203Creutzfeldt-jakob diseaseEnrichmentPRNP1.62
204Hereditary methemoglobinemiaEnrichmentCYB5A1.62
205X-linked emery-dreifuss muscular dystrophyEnrichmentEMD1.62
206Mitochondrial dna depletion syndromeEnrichmentMPV171.56
207Familial hypercholanemiaEnrichmentBAAT1.56
208Multiple enchondromatosis, maffucci typeEnrichmentIDH11.49
209Paroxysmal dystoniaEnrichmentPEX61.49
210Adrenal hyperplasia, congenital, due to 17-alpha-hydroxylase deficiencyEnrichmentCYB5A1.45
211Deafness, autosomal recessive 3EnrichmentOTOF1.45
212Glioma susceptibility 1EnrichmentIDH11.44
213Isolated atp synthase deficiencyEnrichmentATP5F1A1.44
214Auditory neuropathyEnrichmentOTOF, TIMM8A1.42
215NephrocalcinosisEnrichmentAGXT1.34
216NephrolithiasisEnrichmentAGXT1.34
217Frontotemporal dementia and/or amyotrophic lateral sclerosis 1EnrichmentCHCHD101.34
218Peripheral nervous system diseaseEnrichmentGDAP11.34
219NeuropathyEnrichmentGDAP11.34
220Emery-dreifuss muscular dystrophy 1, x-linkedEnrichmentEMD1.33
221Frontotemporal dementia 2EnrichmentPRNP1.33
222Emery-dreifuss muscular dystrophyEnrichmentEMD1.33
223Movement diseaseEnrichmentBCS1L1.30
224Lactic acidosisEnrichmentATP5F1A1.26
225Cystic fibrosisEnrichmentHMOX1, STX1A1.25
226Deafness, autosomal recessive 9EnrichmentOTOF1.23
227Otof-related hearing lossEnrichmentOTOF1.23
228Hypercholesterolemia, familial, 1EnrichmentEPHX21.23
229Premature menopauseEnrichmentPEX61.23
230Charcot-marie-tooth diseaseEnrichmentGDAP11.23
231Mitochondrial diseaseEnrichmentGFER, TIMM501.21
232Isolated macular dystrophyEnrichmentACO21.20
233Familial hypercholesterolemiaEnrichmentEPHX21.17
234GliosarcomaEnrichmentIDH11.14
235HypertensionEnrichmentMTX21.14
236Giant cell glioblastomaEnrichmentIDH11.12
237Alzheimer's disease 1EnrichmentAPP1.09
238LeukodystrophyEnrichmentHSPD11.05
239Focal segmental glomerulosclerosisEnrichmentCOQ21.05
240Retinitis pigmentosaEnrichmentPHYH1.04
241Nephrotic syndromeEnrichmentAGXT, COQ21.03
242MalariaEnrichmentNOS20.99
243Developmental and epileptic encephalopathy 1EnrichmentSLC25A120.96
244Hereditary retinal dystrophyEnrichmentPHYH0.91
245Fundus dystrophyEnrichmentPHYH0.91
246Familial isolated dilated cardiomyopathyEnrichmentGET3, TAFAZZIN0.88
247Connective tissue diseaseEnrichmentPEX70.86
248Pulmonary disease, chronic obstructiveEnrichmentHMOX10.84
249Alzheimer's diseaseEnrichmentAPP0.84
250Left ventricular noncompactionEnrichmentTAFAZZIN0.82
251Frontotemporal dementia and/or amyotrophic lateral sclerosis 7EnrichmentCHCHD10, DAO0.82
252Non-syndromic x-linked intellectual disabilityEnrichmentUSP9X0.81
253Leukemia, acute myeloidEnrichmentIDH10.77
254Hypertrophic cardiomyopathyEnrichmentPMPCA0.74
255Alzheimer disease, familial, 1EnrichmentAPP0.73
256West syndromeEnrichmentSLC25A120.73
257Neuromuscular diseaseEnrichmentEMD0.71
258Sensorineural hearing lossEnrichmentPEX60.70
259Neuropathy, hereditary motor and sensory, okinawa typeEnrichmentEMD0.69
260Williams-beuren syndromeEnrichmentSTX1A0.67
261Spastic ataxiaEnrichmentPEX100.66
262Primary ovarian insufficiencyEnrichmentPEX60.62
263Dilated cardiomyopathyEnrichmentEMD, TAFAZZIN0.61
264Ear malformationEnrichmentOTOF0.60
265Leigh syndrome, nuclearEnrichmentBCS1L0.56
266Leigh diseaseEnrichmentBCS1L0.52
267Non-syndromic genetic deafnessEnrichmentOTOF0.45
268MyopathyEnrichmentEMD0.42
269Nonsyndromic hearing lossEnrichmentOTOF0.40
270MicrocephalyEnrichmentBCS1L0.36
271Deafness, autosomal recessiveEnrichmentOTOF0.27
272Autosomal recessive nonsyndromic deafnessEnrichmentOTOF0.27
273AutismEnrichmentSTX1A0.24
274Rare autosomal recessive non-syndromic sensorineural deafness type dfnbEnrichmentOTOF0.18

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