ERBB3 Chromosome 12

Erb-b2 receptor tyrosine kinase 3
18 variants 18 Health Risk

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What This Gene Does
This gene encodes a member of the epidermal growth factor receptor (EGFR) family of receptor tyrosine kinases. This membrane-bound protein has a neuregulin binding domain but not an active kinase domain. It therefore can bind this ligand but not convey the signal into the cell through protein phosphorylation. However, it does form heterodimers with other EGF receptor family members which do have kinase activity. Heterodimerization leads to the activation of pathways which lead to cell proliferation or differentiation. Amplification of this gene and/or overexpression of its protein have been reported in numerous cancers, including prostate, bladder, and breast tumors. Alternate transcriptional splice variants encoding different isoforms have been characterized. One isoform lacks the intermembrane region and is secreted outside the cell. This form acts to modulate the activity of the membrane-bound form. Additional splice variants have also been reported, but they have not been thoroughly characterized. [provided by RefSeq, Jul 2008]
Gene Info
Gene Group
Erb-b2 receptor tyrosine kinases
Locus Type
gene with protein product
Location
12q13.2
Ensembl
ENSG00000065361
Associated Conditions (10)
ERBB3-related disorder
Erythroleukemia
familial
susceptibility to
Visceral neuropathy
1
autosomal recessive
Lethal congenital contracture syndrome 2
Malignant tumor of urinary bladder
Neoplasm
Key Variants
All Variants (18)
RSID Category Clinical Significance Conditions
RS56017157 Health Risk Conflicting classifications of pathogenicity ERBB3-related disorder, Erythroleukemia, familial
RS56259600 Health Risk Conflicting classifications of pathogenicity Erythroleukemia, familial, susceptibility to
RS755855285 Health Risk Conflicting classifications of pathogenicity Erythroleukemia, familial, susceptibility to
RS1057519169 Health Risk Likely pathogenic
RS141230043 Health Risk Likely pathogenic Lethal congenital contracture syndrome 2, Lethal congenital contracture syndrome 2
RS1565859132 Health Risk Likely pathogenic Lethal congenital contracture syndrome 2, Lethal congenital contracture syndrome 2
RS2136818517 Health Risk Likely pathogenic Visceral neuropathy, familial, 1
RS2136822199 Health Risk Likely pathogenic Visceral neuropathy, familial, 1
RS2540758459 Health Risk Likely pathogenic ERBB3-related disorder, ERBB3-related disorder
RS762129530 Health Risk Likely pathogenic Lethal congenital contracture syndrome 2, Lethal congenital contracture syndrome 2
RS771935902 Health Risk Likely pathogenic ERBB3-related disorder, ERBB3-related disorder
RS1057519893 Health Risk Pathogenic Malignant tumor of urinary bladder, Neoplasm, Malignant tumor of urinary bladder
RS146486757 Health Risk Pathogenic Malignant tumor of urinary bladder, Malignant tumor of urinary bladder
RS1869082532 Health Risk Pathogenic Visceral neuropathy, familial, 1
RS2136815265 Health Risk Pathogenic Visceral neuropathy, familial, 1
RS2136822757 Health Risk Pathogenic Malignant tumor of urinary bladder, Malignant tumor of urinary bladder
RS2540742315 Health Risk Pathogenic Malignant tumor of urinary bladder, Malignant tumor of urinary bladder
RS766102716 Health Risk Pathogenic Visceral neuropathy, familial, 1
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