Product Description

RB27A Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES10119-50, ES10119-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

Background: The protein encoded by this gene belongs to the small GTPase superfamily, Rab family. The protein is membrane-bound and may be involved in protein transport and small GTPase mediated signal transduction. Mutations in this gene are associated with Griscelli syndrome type 2. Alternative splicing occurs at this locus and four transcript variants encoding the same protein have been identified. [provided by RefSeq, Jul 2008],

Reactivity: Human, Rat, Mouse

Host: Rabbit

Applications: WB, ELISA

Clonality: Polyclonal

Immunogen: Synthesized peptide derived from human protein. at AA range: 90-170

Isotype: IgG

Target: RB27A

Specificity: RB27A Polyclonal Antibody detects endogenous levels of protein.

Dilution: WB 1:500-2000 ELISA 1:5000-20000

Purification: The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.

Concentration: 1 mg/ml,

Formlation: Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.

Expression: Found in all the examined tissues except in brain, Low expression was found in thymus, kidney, muscle and placenta, Detected in melanocytes, and in most tumor cell lines examined, Expressed in cytotoxic T-lymphocytes (CTL) and mast cells,

Observed Band (KD): 24kD

Function: Disease: Defects in RAB27A are a cause of Griscelli syndrome type-2 (GS2) [MIM: 607624]. Griscelli syndrome is a rare autosomal recessive disorder that results in pigmentary dilution of the skin and hair, the presence of large clumps of pigment in hair shafts, and an accumulation of melanosomes in melanocytes. GS2 patients also develop an uncontrolled T-lymphocyte and macrophage activation syndrome, known as hemophagocytic syndrome, leading to death in the absence of bone marrow transplantation. Neurological impairment is present in some patients, likely as a result of hemophagocytic syndrome
Online Information: RAB27A mutation db,Online Information: Retina International's Scientific Newsletter,Similarity: Belongs to the small GTPase superfamily. Rab family
Subcellular Location: Identified by mass spectrometry in melanosome fractions from stage I to stage IV
Subunit: Binds SYTL1, SYTL2, SLAC

Subcellular Location: Membrane, Lipid-anchor Melanosome Late endosome Lysosome Identified by mass spectrometry in melanosome fractions from stage I to stage IV (PubMed:12643545, PubMed:17081065) Localizes to endosomal exocytic vesicles (PubMed:17237785)

Storage and Stability: -20°C/1 year

Gene Name: RAB27A RAB27

Protein Name: Ras-related protein Rab-27A (Rab-27) (GTP-binding protein Ram)

Mouse SwissProt Number: Q9ERI2

Rat SwissProt Number: P23640

Research Use Only