CPA6 Chromosome 8

Carboxypeptidase A6
18 variants 18 Health Risk

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What This Gene Does
The gene encodes a member of the peptidase M14 family of metallocarboxypeptidases. The encoded preproprotein is proteolytically processed to generate the mature enzyme, which catalyzes the release of large hydrophobic C-terminal amino acids. This enzyme has functions ranging from digestion of food to selective biosynthesis of neuroendocrine peptides. Mutations in this gene may be linked to epilepsy and febrile seizures, and a translocation t(6;8)(q26;q13) involving this gene has been associated with Duane retraction syndrome. [provided by RefSeq, May 2016]
Gene Info
Gene Group
M14 carboxypeptidases
Locus Type
gene with protein product
Location
8q13.2
Ensembl
ENSG00000165078
Associated Conditions (13)
Febrile seizures
familial
11
Intellectual disability
Familial temporal lobe epilepsy 5
CPA6-related disorder
Epilepsy
Global developmental delay
Self-limited epilepsy with centrotemporal spikes
7 conditions
Adrenocortical carcinoma
hereditary
Lung cancer
Key Variants
All Variants (18)
RSID Category Clinical Significance Conditions
RS114402678 Health Risk Conflicting classifications of pathogenicity Febrile seizures, familial, 11
RS138279913 Health Risk Conflicting classifications of pathogenicity Febrile seizures, familial, 11
RS139145929 Health Risk Conflicting classifications of pathogenicity Epilepsy, Febrile seizures, familial
RS139178030 Health Risk Conflicting classifications of pathogenicity Febrile seizures, familial, 11
RS143107403 Health Risk Conflicting classifications of pathogenicity Familial temporal lobe epilepsy 5, Febrile seizures, familial
RS143321447 Health Risk Conflicting classifications of pathogenicity Familial temporal lobe epilepsy 5, Febrile seizures, familial
RS147046973 Health Risk Conflicting classifications of pathogenicity Febrile seizures, familial, 11
RS147067921 Health Risk Conflicting classifications of pathogenicity Febrile seizures, familial, 11
RS183899632 Health Risk Conflicting classifications of pathogenicity Febrile seizures, familial, 11
RS201265791 Health Risk Conflicting classifications of pathogenicity Febrile seizures, familial, 11
RS201643526 Health Risk Conflicting classifications of pathogenicity Familial temporal lobe epilepsy 5, Febrile seizures, familial
RS35993949 Health Risk Conflicting classifications of pathogenicity Familial temporal lobe epilepsy 5, Global developmental delay, Self-limited epilepsy with centrotemporal spikes
RS368736137 Health Risk Conflicting classifications of pathogenicity Familial temporal lobe epilepsy 5, Febrile seizures, familial
RS372964579 Health Risk Conflicting classifications of pathogenicity Febrile seizures, familial, 11
RS61738009 Health Risk Conflicting classifications of pathogenicity Familial temporal lobe epilepsy 5, Global developmental delay, Self-limited epilepsy with centrotemporal spikes
RS752233230 Health Risk Conflicting classifications of pathogenicity Febrile seizures, familial, 11
RS778807405 Health Risk Conflicting classifications of pathogenicity Febrile seizures, familial, 11
RS773734224 Health Risk Likely pathogenic
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