CCS Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	WB
Recommend Dilution	Western Blot: 1/500 - 1/1000.
Reactivity	Human
Host	Rabbit
Clonality	Polyclonal
Specificity	This antibody detects endogenous levels of CCCS protein. (region surrounding Pro271)
Formulation	Phosphate buffered saline (PBS), pH 7.2. State: Aff - Purified State: Liquid purified Ig fraction Preservative: 0.05% sodium azide
Concentration	1.0 mg/ml
Purification	Affinity chromatography (> 95% (by SDS-PAGE)
Conjugation	Unconjugated
Storage Condition	Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing.
Predicted Protein Size	~ 32 kDa
Gene Name	copper chaperone for superoxide dismutase
Database Link	Entrez Gene 9973 Human UniProt ID O14618
Background	Cu-Zn superoxide dismutase-1 (SOD-1) is a well characterized cytosolic scavenger of oxygen free radicals that requires copper and zinc binding to potentiate its enzymatic activity. Copper chaperone for SOD-1 (CCS) is essential for the incorporation of copper into SOD-1, and therefore is necessary for its enzymatic activity. CCS prevents copper ions from binding to intracellular copper scavengers and provides the SOD-1 enzyme with the necessary copper cofactor. CCS escorts copper only to SOD-1 and fails to deliver copper to proteins in the mitochondria, nucleus or secretory pathway. CCS interacts with both wildtype and mutated forms of SOD-1 through CCS domains that are homologous in SOD-1. CCS exists as a homodimer that may form a heterodimer with SOD-1 during copper loading. While many tissues express CCS, the chaperone is most abundant in the kidney, liver and Purkinje cells in the neuropil of the central nervous system. The gene for CCS maps to human chromosome 11q13.
Synonyms	Superoxide Dismutase Copper Chaperone, SOD-4
Reference Data
Protein Families	Druggable Genome
Protein Pathways	Amyotrophic lateral sclerosis (ALS)

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