PRODH Chromosome 22
Proline dehydrogenase 1
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What This Gene Does
This gene encodes a mitochondrial protein that catalyzes the first step in proline degradation. Mutations in this gene are associated with hyperprolinemia type 1 and susceptibility to schizophrenia 4 (SCZD4). This gene is located on chromosome 22q11.21, a region which has also been associated with the contiguous gene deletion syndromes, DiGeorge and CATCH22. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Aug 2010]
Gene Info
Gene Group
Flavoproteins
Locus Type
gene with protein product
Location
22q11.21
Ensembl
ENSG00000100033
Associated Conditions (5)
Proline dehydrogenase deficiency
Schizophrenia 4
Inborn genetic diseases
Acute myeloid leukemia
Uterine corpus endometrial carcinoma
Key Variants
RS1076466
Conflicting classifications of pathogenicity
Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency
Health Risk
RS1210314134
Conflicting classifications of pathogenicity
Proline dehydrogenase deficiency, Proline dehydrogenase deficiency
Health Risk
RS1325076439
Conflicting classifications of pathogenicity
Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency
Health Risk
RS137852934
Conflicting classifications of pathogenicity
Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency
Health Risk
RS139870359
Conflicting classifications of pathogenicity
Proline dehydrogenase deficiency, Inborn genetic diseases, Proline dehydrogenase deficiency
Health Risk
RS142346005
Conflicting classifications of pathogenicity
Inborn genetic diseases, Proline dehydrogenase deficiency, Schizophrenia 4
Health Risk
RS148375080
Conflicting classifications of pathogenicity
Proline dehydrogenase deficiency, Proline dehydrogenase deficiency
Health Risk
RS150288279
Conflicting classifications of pathogenicity
Inborn genetic diseases, Proline dehydrogenase deficiency, Inborn genetic diseases
Health Risk
RS1807467
Conflicting classifications of pathogenicity
Schizophrenia 4, Proline dehydrogenase deficiency, Inborn genetic diseases
Health Risk
RS184218784
Conflicting classifications of pathogenicity
Proline dehydrogenase deficiency, Schizophrenia 4, Inborn genetic diseases
Health Risk
RS2870984
Conflicting classifications of pathogenicity
Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency
Health Risk
RS2904551
Conflicting classifications of pathogenicity
Schizophrenia 4, Proline dehydrogenase deficiency, Inborn genetic diseases
Health Risk
All Variants (33)
| RSID | Category | Clinical Significance | Conditions |
|---|---|---|---|
| RS1076466 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency |
| RS1210314134 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS1325076439 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency |
| RS137852934 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency |
| RS139870359 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Inborn genetic diseases, Proline dehydrogenase deficiency |
| RS142346005 | Health Risk | Conflicting classifications of pathogenicity | Inborn genetic diseases, Proline dehydrogenase deficiency, Schizophrenia 4 |
| RS148375080 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS150288279 | Health Risk | Conflicting classifications of pathogenicity | Inborn genetic diseases, Proline dehydrogenase deficiency, Inborn genetic diseases |
| RS1807467 | Health Risk | Conflicting classifications of pathogenicity | Schizophrenia 4, Proline dehydrogenase deficiency, Inborn genetic diseases |
| RS184218784 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Schizophrenia 4, Inborn genetic diseases |
| RS2870984 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency |
| RS2904551 | Health Risk | Conflicting classifications of pathogenicity | Schizophrenia 4, Proline dehydrogenase deficiency, Inborn genetic diseases |
| RS2904552 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency |
| RS372187772 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency |
| RS3970555 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency |
| RS3970559 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency |
| RS535186636 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS539772713 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS747609336 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS751149776 | Health Risk | Conflicting classifications of pathogenicity | Proline dehydrogenase deficiency, Schizophrenia 4, Acute myeloid leukemia |
| RS2082011693 | Health Risk | Likely pathogenic | Schizophrenia 4, Proline dehydrogenase deficiency, Schizophrenia 4 |
| RS2870997 | Health Risk | Likely pathogenic | Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency |
| RS1305005935 | Health Risk | Pathogenic | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS1347601186 | Health Risk | Pathogenic | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS193919334 | Health Risk | Pathogenic | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS2146223995 | Health Risk | Pathogenic | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS2517453236 | Health Risk | Pathogenic | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS2517455884 | Health Risk | Pathogenic | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS2517460527 | Health Risk | Pathogenic | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS2517463579 | Health Risk | Pathogenic | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS755378169 | Health Risk | Pathogenic | Proline dehydrogenase deficiency, Proline dehydrogenase deficiency |
| RS772562722 | Health Risk | Pathogenic | Inborn genetic diseases, Proline dehydrogenase deficiency, Inborn genetic diseases |
| RS780557421 | Health Risk | Pathogenic/Likely pathogenic | Proline dehydrogenase deficiency, Schizophrenia 4, Proline dehydrogenase deficiency |