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Key Documents

SAB5702658

Sigma-Aldrich

Anti-CHM Antibody, clone 2F7W2, Rabbit Monoclonal

Synonym(s):

CHM, DXS540, GGTA, HSD-32, REP-1, TCD, CHM, Rab escort protein 1

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About This Item

UNSPSC Code:
12352203

biological source

rabbit

Quality Level

material

colorless

clone

2F7W2, monoclonal

form

liquid

mol wt

101 kDa

species reactivity

human

concentration

0.40 mg/mL

technique(s)

western blot: 1:500 - 1:2000

color

colorless

isotype

IgG

immunogen sequence

MADTLPSEFDVIVIGTGLPESIIAAACSRSGRRVLHVDSRSYYGGNWASFSFSGLLSWLKEYQENSDIVSDSPVWQDQILENEEAIALSRKDKTIQHVEV

UniProt accession no.

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... CHM(1121)

General description

This gene encodes component A of the RAB geranylgeranyl transferase holoenzyme. In the dimeric holoenzyme, this subunit binds unprenylated Rab GTPases and then presents them to the catalytic Rab GGTase subunit for the geranylgeranyl transfer reaction. Rab GTPases need to be geranylgeranyled on either one or two cysteine residues in their C-terminus to localize to the correct intracellular membrane. Mutations in this gene are a cause of choroideremia; also known as tapetochoroidal dystrophy (TCD). This X-linked disease is characterized by progressive dystrophy of the choroid, retinal pigment epithelium and retina. Alternatively spliced transcript variants have been found for this gene.

Immunogen

A synthetic peptide corresponding to a sequence within amino acids 1-100 of human CHM (P24386).
PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.

Application

WB

Storage Class

10 - Combustible liquids

wgk_germany

WGK 1


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