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CHM Antibody - N-terminal region

Product Specifications

CAS Number

9007-83-4

Gene Name

Choroideremia (Rab escort protein 1)

Gene Aliases

TCD, GGTA, REP-1, DXS540, HSD-32

Gene ID

1121

Reactivity

Human

Immunogen

Purified recombinant peptide derived from within residues 1-80 aa at the N-terminus of rat Rep1 produced in E. coli.

Target

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. Alternative splicing results in multiple transcript variants encoding different isoforms.

Clonality

Polyclonal

Conjugation

Unconjugated

Type

Polyclonal Antibody

Applications

WB

Purification

Epitope-affinity purified from goat antiserum.

Assay Protocol

Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Reconstitution & Storage Instructions Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Antibody Array (AA) Protocol

Concentration

3 mg/ml

Homology

Mouse, rat, bovine, canine, chicken/avian, donkey, feline, goat, guinea pig, hamster, horse, porcine, rabbit, sheep, simian

Format

Liquid

Reconstitution

For continuous use, store at 2-8 C for one-two days. For extended storage, store in -20 C freezer. Working dilution samples should be discarded if not used within 12 hours.

Fragment

IgG

Specificity

Choroideremia (Rab escort protein 1)

NCBI Gene Symbol

CHM

Host or Source

Goat

Gene Name URL

CHM

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