GPHN Chromosome 14
Gephyrin
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What This Gene Does
This gene encodes a neuronal assembly protein that anchors inhibitory neurotransmitter receptors to the postsynaptic cytoskeleton via high affinity binding to a receptor subunit domain and tubulin dimers. In nonneuronal tissues, the encoded protein is also required for molybdenum cofactor biosynthesis. Mutations in this gene may be associated with the neurological condition hyperplexia and also lead to molybdenum cofactor deficiency. Numerous alternatively spliced transcript variants encoding different isoforms have been described; however, the full-length nature of all transcript variants is not currently known. [provided by RefSeq, Jul 2008]
Associated Conditions (8)
Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C
Hyperekplexia 1
Inborn genetic diseases
Seizure
Hepatocellular carcinoma
Thyroid cancer
nonmedullary
1
Key Variants
RS117256383
Conflicting classifications of pathogenicity
Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Hyperekplexia 1, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C
Health Risk
RS140021399
Conflicting classifications of pathogenicity
Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Inborn genetic diseases, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C
Health Risk
RS144247888
Conflicting classifications of pathogenicity
Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Inborn genetic diseases, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C
Health Risk
RS1567108397
Conflicting classifications of pathogenicity
Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C
Health Risk
RS2079054125
Conflicting classifications of pathogenicity
Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Inborn genetic diseases, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C
Health Risk
RS529026517
Conflicting classifications of pathogenicity
Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C
Health Risk
RS550187024
Conflicting classifications of pathogenicity
Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Inborn genetic diseases, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C
Health Risk
RS774132595
Conflicting classifications of pathogenicity
Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Inborn genetic diseases, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C
Health Risk
RS776988756
Conflicting classifications of pathogenicity
Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Inborn genetic diseases, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C
Health Risk
RS2066910297
Likely pathogenic
Seizure, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Seizure
Health Risk
RS2077665118
Likely pathogenic
Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C
Health Risk
RS2082439915
Likely pathogenic
Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Hepatocellular carcinoma, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C
Health Risk
All Variants (46)
| RSID | Category | Clinical Significance | Conditions |
|---|---|---|---|
| RS117256383 | Health Risk | Conflicting classifications of pathogenicity | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Hyperekplexia 1, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS140021399 | Health Risk | Conflicting classifications of pathogenicity | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Inborn genetic diseases, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS144247888 | Health Risk | Conflicting classifications of pathogenicity | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Inborn genetic diseases, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS1567108397 | Health Risk | Conflicting classifications of pathogenicity | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2079054125 | Health Risk | Conflicting classifications of pathogenicity | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Inborn genetic diseases, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS529026517 | Health Risk | Conflicting classifications of pathogenicity | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS550187024 | Health Risk | Conflicting classifications of pathogenicity | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Inborn genetic diseases, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS774132595 | Health Risk | Conflicting classifications of pathogenicity | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Inborn genetic diseases, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS776988756 | Health Risk | Conflicting classifications of pathogenicity | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Inborn genetic diseases, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2066910297 | Health Risk | Likely pathogenic | Seizure, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Seizure |
| RS2077665118 | Health Risk | Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2082439915 | Health Risk | Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Hepatocellular carcinoma, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2140003018 | Health Risk | Likely pathogenic | Thyroid cancer, nonmedullary, 1 |
| RS2153685300 | Health Risk | Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2542172605 | Health Risk | Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2543060389 | Health Risk | Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2543395293 | Health Risk | Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2543402379 | Health Risk | Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2543402445 | Health Risk | Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2549068048 | Health Risk | Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2549284450 | Health Risk | Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2549499286 | Health Risk | Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2549615253 | Health Risk | Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS533003321 | Health Risk | Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS1214630241 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS1352910925 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS1595241291 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2061323841 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2153668269 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2153668310 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2153717733 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2543059711 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2543059971 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2543230821 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2543402851 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2543525337 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2543888962 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2549068250 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2549597698 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2549615369 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2549615601 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2549619648 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2549619766 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2549782519 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS397518420 | Health Risk | Pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |
| RS2153561495 | Health Risk | Pathogenic/Likely pathogenic | Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C, Sulfite oxidase deficiency due to molybdenum cofactor deficiency type C |