Code
OSR00306W
$240USD
ID Tag
Rb668-060908-WS
Immunogen
A synthetic peptide from aa region 160-205 of human RAB7 conjugated to blue carrier protein has been used as the antigen. The peptide is homologous in mouse and xenopus.
Accession
Also known
RAB7A, RAB7
Target
FUNCTION: Involved in late endocytic transport. Contributes to the maturation of phagosomes (acidification).
SUBCELLULAR LOCATION: Late endosome. Lysosome. Cytoplasmic vesicle, phagosome. Melanosome. Note: Identified by mass spectrometry in melanosome fractions from stage I to stage IV.
TISSUE SPECIFICITY: Widely expressed; high expression found in skeletal muscle.
DISEASE: Defects in RAB7A are the cause of Charcot-Marie-Tooth disease type 2B (CMT2B) also known as hereditary motor and sensory neuropathy II (HMSN2). CMT2B is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. CMT2B is clinically characterized by marked distal muscle weakness and a high frequency of foot ulcers, infections and amputations of the toes. CMT2B inheritance is autosomal dominant.
Storage
Maintain the lyophilised/reconstituted antibodies frozen at -20C for long term storage and refrigerated at 2-8C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.
Expiry Date
12 months after reconstitution
Shipping
This item will be shipped to you at ambient temperature in a lyophilised form.
References
1. Vitelli R, et al. Biochem. Biophys. Res. Commun. 229:887-890(1996).
2. Ota T, et al. Nat. Genet. 36:40-45(2004).
Limitation
For research use only
Code
OSR00306W
$240USD
Unit size
100 ul
Conjugate
Unconjugated antibody
Host
NZ white rabbit
Purity
Whole serum
Clonality
Polyclonal
Isotype
Polyclonal, whole serum
Applications
IHC. Use at a dilution of 1:300 to 1: 2000. The optimal dilution should be determined by the end user. Not yet tested in other applications.
Specificity
Specific for RAB7.
Spcs X-react.
Human, mouse. Other species not yet tested.
Format
Lyophilised
Reconstitution
Reconstitute in 100 ul of sterile water. Centrifuge to remove any insoluble material.