Unfolded Protein Response (UPR)

Pathway network for the Unfolded Protein Response (UPR) SuperPath

Sources:
  • Reactome

Pathways in the Unfolded Protein Response (UPR) SuperPath

#NameSourceGenes
1Unfolded Protein Response (UPR)Reactome
2IRE1alpha activates chaperonesReactome
3XBP1(S) activates chaperone genesReactome
4ATF6 (ATF6-alpha) activates chaperonesReactome
5ATF6 (ATF6-alpha) activates chaperone genesReactome
6CREB3 factors activate genesReactome
7ATF6B (ATF6-beta) activates chaperonesReactome

Gene overlap in member pathways for Unfolded Protein Response (UPR) SuperPath

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

Disorders associated with Unfolded Protein Response (UPR) SuperPath

according to GeneCards Suite gene sharing
#DisorderTypeGenesScore
1MyxofibrosarcomaEnrichmentCREB3L1, CREB3L25.93
2Pontocerebellar hypoplasia, type 1eEnrichmentEXOSC3, EXOSC8, EXOSC95.21
3Osteogenesis imperfecta, type iiiEnrichmentCREB3L1, MBTPS24.39
4Olmsted syndrome, x-linkedEnrichmentMBTPS23.53
5Osteogenesis imperfecta, type xixEnrichmentMBTPS23.53
6Keratosis follicularis spinulosa decalvans, x-linkedEnrichmentMBTPS23.53
7Cataract, alopecia, oral mucosal disorder, and psoriasis-like syndromeEnrichmentMBTPS13.53
8Ifap syndromeEnrichmentMBTPS23.53
9Bresek syndromeEnrichmentMBTPS23.53
10Congenital muscular dystrophyEnrichmentFKBP14, LMNA3.47
11Spondyloepiphyseal dysplasia, kondo-fu typeEnrichmentMBTPS13.23
12Osteogenesis imperfecta, type xviEnrichmentCREB3L13.18
13Hypertriglyceridemia 2EnrichmentCREB3L33.18
14Major affective disorder 7EnrichmentXBP13.13
15Achromatopsia 7EnrichmentATF63.13
16Ifap syndrome 1, with or without bresheck syndromeEnrichmentMBTPS23.05
17Keratosis follicularis spinulosa decalvansEnrichmentMBTPS23.05
18Mutilating palmoplantar keratoderma with periorificial keratotic plaquesEnrichmentMBTPS23.05
19Transient infantile hypertriglyceridemia and hepatosteatosisEnrichmentCREB3L32.88
20Osteogenesis imperfecta, type iEnrichmentMBTPS22.75
21Thrombocythemia 1EnrichmentCALR2.66
22Myxoid liposarcomaEnrichmentDDIT32.66
23Hypertriglyceridemia 1EnrichmentCREB3L32.58
24Budd-chiari syndromeEnrichmentCALR2.53
25Cataract 41EnrichmentWFS12.45
26Perry syndromeEnrichmentDCTN12.45
27Wolfram syndrome 1EnrichmentWFS12.45
28Deafness, autosomal dominant 6EnrichmentWFS12.45
29Spinocerebellar ataxia, autosomal recessive 7EnrichmentTPP12.45
30Wolfram-like syndrome, autosomal dominantEnrichmentWFS12.45
31Halperin-birk syndromeEnrichmentSEC31A2.45
32Immunoskeletal dysplasia with neurodevelopmental abnormalitiesEnrichmentEXTL32.45
33Neuronopathy, distal hereditary motor, autosomal dominant 14EnrichmentDCTN12.45
34Warsaw breakage syndromeEnrichmentDDX112.45
35Atypical werner syndromeEnrichmentLMNA2.45
36Polycystic kidney disease 6 with or without polycystic liver diseaseEnrichmentDNAJB112.45
37Mandibuloacral dysplasiaEnrichmentLMNA2.45
38Atrioventricular blockEnrichmentLMNA2.45
39Wolfram-like syndromeEnrichmentWFS12.45
40Capillary leak syndromeEnrichmentTLN12.45
41Lmna-related cardiocutaneous progeria syndromeEnrichmentLMNA2.45
42Skeletal dysplasia-t-cell immunodeficiency-developmental delay syndromeEnrichmentEXTL32.45
43Autosomal semi-dominant severe lipodystrophic laminopathyEnrichmentLMNA2.45
44Wfs1 spectrum disorderEnrichmentWFS12.45
45Autosomal recessive axonal hereditary motor and sensory neuropathyEnrichmentLMNA2.45
46LaminopathyEnrichmentLMNA2.45
47Osteogenesis imperfecta, type ivEnrichmentMBTPS22.42
48MyelofibrosisEnrichmentCALR2.29
49Essential thrombocythemiaEnrichmentCALR2.29
50Pontocerebellar hypoplasiaEnrichmentEXOSC3, EXOSC92.18
51Dyskeratosis congenita, autosomal recessive 6EnrichmentPARN2.16
52Pontocerebellar hypoplasia, type 1fEnrichmentEXOSC12.16
53Mehmo syndromeEnrichmentEIF2S32.16
54Cerebellar ataxia, brain abnormalities, and cardiac conduction defectsEnrichmentEXOSC52.16
55Pulmonary fibrosis and/or bone marrow failure syndrome, telomere-related, 4EnrichmentPARN2.16
56Pontocerebellar hypoplasia, type 1cEnrichmentEXOSC82.16
57Pontocerebellar hypoplasia, type 1dEnrichmentEXOSC92.16
58Short stature, hearing loss, retinitis pigmentosa, and distinctive faciesEnrichmentEXOSC22.16
59Mandibuloacral dysplasia with type a lipodystrophyEnrichmentLMNA2.15
60Ceroid lipofuscinosis, neuronal, 2EnrichmentTPP12.15
61Immunodeficiency 59 and hypoglycemiaEnrichmentHYOU12.15
62Heart-hand syndrome, slovenian typeEnrichmentLMNA2.15
63Charcot-marie-tooth disease, axonal, type 2b1EnrichmentLMNA2.15
64Cardiomyopathy, dilated, with hypergonadotropic hypogonadismEnrichmentLMNA2.15
65Ataxia, combined cerebellar and peripheral, with hearing loss and diabetes mellitusEnrichmentDNAJC32.15
66Myasthenic syndrome, congenital, 12EnrichmentGFPT12.15
67Ceroid lipofuscinosis, neuronal, 3EnrichmentTPP12.15
68Cardiomyopathy, dilated, 1dEnrichmentLMNA2.15
69Restrictive dermopathy 2EnrichmentLMNA2.15
70Emery-dreifuss muscular dystrophy 3, autosomal recessiveEnrichmentLMNA2.15
71Ehlers-danlos syndrome, kyphoscoliotic type, 2EnrichmentFKBP142.15
72Lipodystrophy, familial partial, type 1EnrichmentLMNA2.15
73Wolfram syndromeEnrichmentWFS12.15
74Distal hereditary motor neuropathy type 7EnrichmentDCTN12.15
75Familial partial lipodystrophyEnrichmentLMNA2.15
76Charcot-marie-tooth disease type 2b1EnrichmentLMNA2.15
77AchromatopsiaEnrichmentATF62.13
78Charcot-marie-tooth diseaseEnrichmentDCTN1, LMNA2.04
79Three m syndrome 1EnrichmentCUL71.98
80Heart defects, congenital, and other congenital anomaliesEnrichmentACADVL1.98
81Restrictive dermopathy 1EnrichmentLMNA1.98
82Lipodystrophy, familial partial, type 2EnrichmentLMNA1.98
83Muscular dystrophy, congenital, lmna-relatedEnrichmentLMNA1.98
84Epilepsy, progressive myoclonic, 6EnrichmentGOSR21.98
85Muscular dystrophy, congenital, with or without seizuresEnrichmentGOSR21.98
86Intellectual developmental disorder, autosomal dominant 62EnrichmentACADVL1.98
87Dlg4-related synaptopathyEnrichmentACADVL1.98
88Restrictive dermopathyEnrichmentLMNA1.98
89Epiphyseal dysplasia, multiple, with early-onset diabetes mellitusEnrichmentEIF2AK31.86
90Congenital pontocerebellar hypoplasia type 1EnrichmentEXOSC31.86
91Hutchinson-gilford progeria syndromeEnrichmentLMNA1.85
92Acyl-coa dehydrogenase, very long-chain, deficiency ofEnrichmentACADVL1.85
93Emery-dreifuss muscular dystrophy 1, x-linkedEnrichmentLMNA1.85
94Microtia-anotiaEnrichmentLMNA1.85
95Emery-dreifuss muscular dystrophyEnrichmentLMNA1.85
96Sick sinus syndromeEnrichmentLMNA1.85
97Brittle bone disorderEnrichmentCREB3L11.82
98Autosomal recessive limb-girdle muscular dystrophy type 2bEnrichmentLMNA1.76
99Histiocytoid hemangiomaEnrichmentLMNA1.76
100Genetic motor neuron diseaseEnrichmentDCTN11.76
101Congenital myasthenic syndromes with glycosylation defectEnrichmentGFPT11.76
102Menkes diseaseEnrichmentEIF2AK31.69
103Pontocerebellar hypoplasia, type 1bEnrichmentEXOSC31.69
104Cerebellar diseaseEnrichmentEXOSC31.69
105Asparagine synthetase deficiencyEnrichmentASNS1.69
106Congenital microcephaly - severe encephalopathy - progressive cerebral atrophy syndromeEnrichmentASNS1.69
107Spinocerebellar ataxia, autosomal recessive 29EnrichmentASNS1.69
108Angelman syndromeEnrichmentTPP11.68
109Emery-dreifuss muscular dystrophy 2, autosomal dominantEnrichmentLMNA1.68
110Shwachman-diamond syndrome 1EnrichmentSRPRA1.68
111Bethlem myopathy 1aEnrichmentLMNA1.61
112Polymicrogyria, bilateral perisylvian, x-linkedEnrichmentWFS11.61
113Severe congenital neutropeniaEnrichmentSRPRA1.55
114MyocarditisEnrichmentLMNA1.55
115Arrhythmogenic right ventricular dysplasia, familial, 9EnrichmentLMNA1.50
116Bilateral perisylvian polymicrogyriaEnrichmentWFS11.50
117Cystic kidney diseaseEnrichmentDNAJB111.50
118Amyotrophic lateral sclerosis 1EnrichmentDCTN11.46
119Myasthenic syndrome, congenital, 4c, associated with acetylcholine receptor deficiencyEnrichmentGFPT11.46
120Progressive myoclonus epilepsyEnrichmentGOSR21.46
121Frontotemporal dementia 1EnrichmentDCTN11.42
122Diabetes mellitusEnrichmentWFS11.42
123Pulmonary fibrosisEnrichmentPARN1.39
124Hoyeraal-hreidarsson syndromeEnrichmentPARN1.39
125Cardiac conduction defectEnrichmentLMNA1.38
126Muscular dystrophy, limb-girdle, autosomal recessive 2EnrichmentLMNA1.38
127Inherited isolated arrhythmogenic ventricular dysplasia, biventricular variantEnrichmentLMNA1.38
128Inherited isolated arrhythmogenic cardiomyopathy, dominant-left variantEnrichmentLMNA1.38
129Myoclonic epilepsy of unverricht and lundborgEnrichmentGOSR21.31
130Congenital myasthenic syndromeEnrichmentGFPT11.31
131Inherited isolated arrhythmogenic cardiomyopathy, dominant-right variantEnrichmentLMNA1.31
132Cone-rod dystrophy 2EnrichmentATF61.31
133Spastic ataxiaEnrichmentEXOSC8, WFS11.29
134Neuronal ceroid lipofuscinosisEnrichmentTPP11.29
135Autosomal dominant polycystic kidney diseaseEnrichmentDNAJB111.29
136Arrhythmogenic right ventricular cardiomyopathyEnrichmentLMNA1.26
137Cardiomyopathy, dilated, 1eEnrichmentLMNA1.23
138Neuromuscular diseaseEnrichmentLMNA1.21
139Polycystic kidney diseaseEnrichmentDNAJB111.21
140Early-onset nuclear cataractEnrichmentWFS11.21
141Neuropathy, hereditary motor and sensory, okinawa typeEnrichmentLMNA1.19
142Cardiomyopathy, dilated, 1aEnrichmentLMNA1.14
143Cardiomyopathy, familial hypertrophic, 1EnrichmentLMNA1.09
144Muscular dystrophyEnrichmentLMNA1.09
145Brugada syndromeEnrichmentLMNA1.05
146Long qt syndromeEnrichmentLMNA0.98
147Peripheral nervous system diseaseEnrichmentLMNA0.97
148NeuropathyEnrichmentLMNA0.97
149Interstitial lung disease 2EnrichmentPARN0.96
150Human immunodeficiency virus type 1EnrichmentCCL20.93
151Left ventricular noncompactionEnrichmentLMNA0.93
152Non-syndromic genetic deafnessEnrichmentWFS10.92
153Congenital myopathyEnrichmentEXOSC30.91
154Dyskeratosis congenitaEnrichmentPARN0.91
155MyopathyEnrichmentACADVL0.88
156LissencephalyEnrichmentEXOSC30.87
157Type 2 diabetes mellitusEnrichmentWFS10.86
158Nonsyndromic hearing lossEnrichmentWFS10.86
159Optic atrophy plus syndromeEnrichmentWFS10.84
160Congenital nervous system abnormalityEnrichmentEXOSC3, TPP10.78
161Nervous system diseaseEnrichmentEXOSC3, TPP10.78
162Rare autosomal dominant non-syndromic sensorineural deafness type dfnaEnrichmentWFS10.77
163Familial isolated dilated cardiomyopathyEnrichmentLMNA0.77
164Frontotemporal dementia and/or amyotrophic lateral sclerosis 7EnrichmentDCTN10.73
165Hereditary retinal dystrophyEnrichmentATF60.71
166Fundus dystrophyEnrichmentATF60.71
167Deafness, autosomal recessiveEnrichmentGOSR20.70
168Autosomal recessive nonsyndromic deafnessEnrichmentGOSR20.69
169Fetal akinesia deformation sequence 1EnrichmentEXOSC30.65
170Breast cancerEnrichmentSHC10.63
171Rare genetic deafnessEnrichmentWFS10.61
172Dilated cardiomyopathyEnrichmentLMNA0.61
173Rare autosomal recessive non-syndromic sensorineural deafness type dfnbEnrichmentGOSR20.57
174Hereditary breast ovarian cancer syndromeEnrichmentHERPUD10.50
175Myeloma, multipleEnrichmentDIS30.49
176MicrocephalyEnrichmentEXOSC30.24

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