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Gene | VHL |
Variant | Q164R |
Impact List | missense |
Protein Effect | loss of function - predicted |
Gene Variant Descriptions | VHL Q164R lies within the Elongin BC complex-interacting region of the Vhl protein (UniProt.org). Q164R retains the ability to bind to the Elongin BC complex and ubiquitinate Hif1a in in vitro assays (PMID: 26503325), however, is predicted to lead to a loss of Vhl protein function as demonstrated by increased ubiquitination by Ube2s leading to reduced Vhl protein stability and impaired ability to adequately regulate Hif protein expression and activity in in vitro assays and cell culture (PMID: 26503325). |
Associated Drug Resistance | |
Category Variants Paths |
VHL mutant VHL inact mut VHL Q164R |
Transcript | NM_000551.4 |
gDNA | chr3:g.10149814A>G |
cDNA | c.491A>G |
Protein | p.Q164R |
Source Database | RefSeq |
Genome Build | GRCh38/hg38 |
Transcript | gDNA | cDNA | Protein | Source Database | Genome Build |
---|---|---|---|---|---|
NM_000551 | chr3:g.10149814A>G | c.491A>G | p.Q164R | RefSeq | GRCh38/hg38 |
NM_000551.3 | chr3:g.10149814A>G | c.491A>G | p.Q164R | RefSeq | GRCh38/hg38 |
NM_000551.4 | chr3:g.10149814A>G | c.491A>G | p.Q164R | RefSeq | GRCh38/hg38 |
Molecular Profile | Indication/Tumor Type | Response Type | Therapy Name | Approval Status | Evidence Type | Efficacy Evidence | References |
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