Anti-CHM Antibody Picoband®

About this product

Boster Bio Anti-CHM Antibody Picoband® catalog # A00814-1. Tested in ELISA, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Specifications

Catalog number

A00814-1

Size / count

100 µg/Vial

Dry ice

No

Hazardous

No

Discontinued

No

Background

Rab escort protein 1 (REP1) also known as rab proteins geranylgeranyltransferase component A 1 is an enzyme that in humans is encoded by the CHM gene. 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.

Synonyms

Rab proteins geranylgeranyltransferase component A 1; Choroideremia protein; Rab escort protein 1; REP-1; TCD protein; CHM; REP1, TCD

Gene Name

CHM

Gene ID

1121

UniProt

P24386

Host

Rabbit

Reactivity

Human, Mouse, Rat

Cross Reactivity

No cross-reactivity with other proteins.

Immunogen

E. coli-derived human CHM recombinant protein (Position: A193-Q380) .

Clonality

Polyclonal

Tissue Specificity

Expressed in monocytes and mature macrophages such as Kupffer cells in the liver, red pulp macrophages in the spleen, cortical macrophages in the thymus, resident bone marrow macrophages and meningeal macrophages of the central nervous system. Expressed also in blood. Isoform 1 is the lowest abundant in the blood. Isoform 2 is the lowest abundant in the liver and the spleen. Isoform 3 is the predominant isoform detected in the blood.

Applications

WB, ELISA

Field of Research

Chaperones, Neuroscience, Neurotransmission, Protein Trafficking, Secretory Vesicles, Signal Transduction, Vesicle Transport

Purification

Immunogen affinity purified.

Concentration

Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Form

Lyophilized

Reconstitution

Add 0.2ml of distilled water will yield a concentration of 500ug/ml.

Function

Substrate-binding subunit of the Rab geranylgeranyltransferase (GGTase) complex. Binds unprenylated Rab proteins and presents the substrate peptide to the catalytic component B composed of RABGGTA and RABGGTB, and remains bound to it after the geranylgeranyl transfer reaction. The component A is thought to be regenerated by transferring its prenylated Rab back to the donor membrane. Besides, a pre-formed complex consisting of CHM and the Rab GGTase dimer (RGGT or component B) can bind to and prenylate Rab proteins; this alternative pathway is proposed to be the predominant pathway for Rab protein geranylgeranylation.

References & Citations

1. Entrez Gene: CHM choroideremia (Rab escort protein 1) . 2. Molloy CM, van de Pol TJ, Brohet RM, Ropers HH, Cremers FP (May 1992) . Three RFLPs for pZ11 (DXS540) in the choroideremia gene at Xq21.2. Nucleic Acids Res. 20 (6) : 1434.

Storage Conditions

Store at -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freeze-thaw cycles.

Calculated Molecular Weight

53384 MW

Observed Molecular Weight

73 kDa

Specificity

No cross reactivity with other proteins.

Gene Name Synonym

CHM, Rab escort protein 1

Subcellular Location

Cytoplasm, cytosol.

Protein Name

Lipopolysaccharide-binding protein

Isotype

Rabbit IgG

Contents

243