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Gene | VHL |
Variant | F136L |
Impact List | missense |
Protein Effect | loss of function |
Gene Variant Descriptions | VHL F136L lies within the CCT complex-binding region and the aromatic tetrahedron core domain of the Vhl protein (UniProt.org, PMID: 23840444). F136L results in decreased Vhl protein stability as compared to wild-type, demonstrating increased misfolding and aggregation of Vhl protein in in-vitro assays (PMID: 23840444), and reduced solubility and decreased binding to Hif1a protein, and overexpression of P136L leads to a lethal phenotype in animal models (PMID: 30194449). |
Associated Drug Resistance | |
Category Variants Paths |
VHL mutant VHL inact mut VHL F136L |
Transcript | NM_000551.4 |
gDNA | chr3:g.10146579T>C |
cDNA | c.406T>C |
Protein | p.F136L |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_000551 | chr3:g.10146579T>C | c.406T>C | p.F136L | RefSeq | GRCh38/hg38 |
NM_000551.4 | chr3:g.10146579T>C | c.406T>C | p.F136L | RefSeq | GRCh38/hg38 |
NM_000551.3 | chr3:g.10146579T>C | c.406T>C | p.F136L | RefSeq | GRCh38/hg38 |
Clinical Trial | Phase | Therapies | Title | Recruitment Status | Covered Countries | Other Countries |
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