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HECW1

Function

E3 ubiquitin-protein ligase that mediates ubiquitination and subsequent degradation of DVL1. Also targets the mutant SOD1 protein involved in familial amyotrophic lateral sclerosis (FALS). Forms cytotoxic aggregates with DVL1, SSR3 and mutant SOD1 that lead to motor neuron death in FALS.

Pathway

Protein modification; protein ubiquitination.

Tissue Specificity

Predominantly expressed in neurons of adult and fetal brain. Weakly expressed in the kidney.

Cellular localization

Alternative names

KIAA0322, NEDL1, HECW1, E3 ubiquitin-protein ligase HECW1, HECT-type E3 ubiquitin transferase HECW1, NEDD4-like E3 ubiquitin-protein ligase 1, hNEDL1

swissprot:Q76N89 omim:610384 entrezGene:23072