E3 ubiquitin-protein ligase HECW1; C2 & WW domain-containing protein 1; NEDD4-like ubiquitin-protein ligase 1; hNEDL1 (HECW1, KIAA0322, NEDL1)

Function

• E3 ubiquitin-protein ligase
• mediates ubiquitination & subsequent degradation of DVL1
• targets mutant SOD1 involved in familial ALS
• ubiquitin conjugation, 3rd step
• interacts with DVL1 & SSR3
• interacts with mutant SOD1

Structure

• contains 1 C2 domain
• contains 1 HECT domains (E6AP-type E3 ubiquitin-protein ligase domain)
• contains 2 WW domains

Compartment

cytoplasm

Expression

• predominantly expressed in neurons of adult & fetal brain
• weakly expressed in the kidney

Pathology

• forms cytotoxic aggregates with DVL1, SSR3 & mutant SOD1 that lead to motor neuron death in familial ALS

More general terms

• E3 ubiquitin ligase

Database

• UniProt: http://www.uniprot.org/uniprot/Q76N89.html