Germline mutations were identified in 300/469 (63%) of the families tested; 137 distinct intragenic germline mutations were detected.
GroupID/ KindredID: 43CA
DiseaseAssertion: VHL
GroupSize: N/A
GroupNumberMales: N/A
GroupNumberFemales: N/A
GroupEthnicity: N/A
GroupAgeRange: N/A
GroupInfo: This germline familial VHL mutation was taken from the Cambridge, Department of Pathology database, with an unknown number of members belonging to Family ID 43CA. Table 3 lists all ID and mutations associated with individuals possessing renal carcinoma, without phechromocytoma.
GroupPresentingHPOs: HP:0005548 (renal cell carcinoma)
GroupHPOFreeText: Described as a VHL families with RCC, without pheochromocytoma. Other phenotypes are likely, however not detailed in this publication
GroupNotHPOs: HP:0002666 (pheochromocytoma)
GroupNotHPOFreeText: N/A
GroupPreviousTesting: N/A
PreviouslyPublished: N/A
SupplementalData: N/A
Variant: p.V66* (c.196delG)
LegacyVariant: p.V66* (c.409delG)
CaseProblemVariantFreeText: N/A
ClinVarID: N/A
CAID: CA432536396 https://reg.clinicalgenome.org/redmine/projects/registry/genboree_registry/allele?hgvsOrDescriptor=NM_000551.3%3Ac.196delG
gnomAD: N/A
VariantEvidence: This paper declares this variant is predicted to compromise the Elongin-binding site by truncating the protein.
MutationType: stop_gained
CivicName: V66* (c.196delG)
MultipleGeneVariants: N/A