Total submissions: 2
Submitter | RCV | SCV | Clinical significance | Condition | Last evaluated | Review status | Method | Comment |
---|---|---|---|---|---|---|---|---|
Labcorp Genetics |
RCV001050973 | SCV001215106 | uncertain significance | Renal carnitine transport defect | 2021-08-31 | criteria provided, single submitter | clinical testing | |
Kasturba Medical College, |
RCV001050973 | SCV005199834 | uncertain significance | Renal carnitine transport defect | criteria provided, single submitter | clinical testing | This variant is reported in ClinVar database (847432). In-silico analysis tools (REVEL, CADD and mutation Taster) predict the variant to be disease-causing and likely to affect the SLC22A5 protein function. Bi-allelic variants in SLC22A5 are associated with carnitine deficiency, systemic primary (MIM#212140) characterized by hypoketotic hypoglycemia, poor feeding, irritability, lethargy, hepatomegaly, elevated liver transaminases, and hyperammonemia. |