NEDL1 is a 1,606 amino acid cytoplasmic protein predominantly expressed in neurons of adult and fetal brain. NEDL1 functions as an E3 ubiquitin-protein ligase that, characteristic of E3 ligase proteins, accepts ubiquitin (in the form of a thioester) from an E2 ubiquitin-conjugating enzyme and transfers that ubiquitin residue to substrates targeted for degradation. NEDL1 mediates ubiquitination and subsequent degradation of Dvl-1 and targets mutant SOD-1. NEDL1 forms cytotoxic aggregates with Dvl, TRAP- and mutant SOD1 that lead to motor neuron death in FALS (familial amyotrophic lateral sclerosis). individuals affect by FALS (also known as Lou Gehrig's disease) experience muscle weakness and atrophy throughout the body. FALS is caused by the degeneration of upper and lower motor neurons resulting in loss of signal to muscles.

NEDL1; C2 and WW domain-containing protein 1; E3 ubiquitin-protein ligase HECW1; HECT; HECT type E3 ubiquitin ligase; HECT, C2 and WW domain containing E3 ubiquitin protein ligase 1; HECT, C2 and WW domain-containing protein 1; HECW 1; Hecw1; HECW1_HUMAN; hNEDL1; NEDD4 like ubiquitin protein ligase 1; NEDD4-like E3 ubiquitin-protein ligase 1; NEDL-1; NEDL 1.

Rat

IHC,IF,ICC

IHC 1:100-500
IF 1:100-500
ICC 1:100-1:400

179kD

23072

Q76N89

https://www.uniprot.org/uniprotkb/Q76N89

Cytoplasm.

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

KLH conjugated synthetic peptide derived from human HECW1

Rabbit

Polyclonal

IgG

affinity purified by Protein A

Liquid

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.