产品名称
RB27A Rabbit Polyclonal Antibody
蛋白名称
Ras-related protein Rab-27A (Rab-27) (GTP-binding protein Ram)
存储缓冲液
Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.
Human Swissprot No.
P51159
Human Swissprot Link
https://www.uniprot.org/uniprotkb/P51159/entry
Mouse Swissprot No.
Q9ERI2
Mouse Swissprot Link
http://www.uniprot.org/uniprot/Q9ERI2
Rat Swissprot Link
http://www.uniprot.org/uniprot/O54941P23640
免疫原
Synthesized peptide derived from human protein . at AA range: 90-170
特异性
RB27A Polyclonal Antibody detects endogenous levels of protein.
稀释度
WB 1:500-2000 ELISA 1:5000-20000
宿主
Polyclonal, Rabbit,IgG
背景介绍
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],
组织表达
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.
细胞定位
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). .
功能
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, SLAC2B, MYRIP, SYTL3, SYTL4 and SYTL5 (By similarity). Binds MLPH.,tissue specificity: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.,
纯化
The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
