Anti-OSTM1 antibody produced in rabbit

Human Protein Atlas Number:

HPA010851    Human Protein Atlas characterization data

Кат. номер

HPA010851-100UL

HPA010851-25UL

OSTM1 (osteopetrosis associated transmembrane protein 1) is one of the genes associated with Autosomal Recessive Osteopetrosis (ARO) in humans. It was first identified in murine model, as the gene associated with grey-lethal mutant mice. This protein is thought to be an E3 ubiquitin ligase as well as a Gα-interacting protein. It is a type II transmembrane protein, which resides in intracellular compartments. In mice, this gene encodes a protein with predicted 338 amino acids. The region between N- and C- termini of the protein is hydrophobic, and shows slight homology to RING-finger proteins. It also has many putative N-linked glycosylation sites. OSTM1 is expressed in multiple tissues such as, osteoclasts, brain, liver and kidney.
Osteopetrosis-associated transmembrane protein 1 precursor recombinant protein epitope signature tag (PrEST)
Anti-OSTM1 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project (www.proteinatlas.org),. Each antibody is tested by immunohistochemistry against hundreds of normal and disease tissues. These images can be viewed on the Human Protein Atlas (HPA) site by clicking on the Image Gallery link. The antibodies are also tested using immunofluorescence and western blotting. To view these protocols, and other useful information about Prestige Antibodies and the HPA, visit sigma.com/prestige,.
Biochem/physiol Actions
The exact function of OSTM1 (osteopetrosis associated transmembrane protein 1) gene is not yet known. However, it acts as a β subunit for the protein ClC-7 (Chloride channel 7), as it co-localizes with ClC-7 in lysosomes and late endosomes in multiple tissues, as well as in the bone-resorbing osteoclasts, in their ruffled borders. This gene is essential for the maturation and normal function of osteocytes and melanocytes. In humans, mutations in this gene lead to autosomal recessive osteopetrosis (ARO). ARO is a rare hereditary disorder, which is a result of oscteoclast resorption failure, and is characterized by bone marrow failure, increased bone density and fractures. OSTM1 is also responsible for normal neural development, and mutations in this gene lead to ARO with severe neuronopathy. This phenotype is characterized by malformations in the central nervous system, blindness, seizures, and deafness.
Features and Benefits
Prestige Antibodies® are highly characterized and extensively validated antibodies with the added benefit of all available characterization data for each target being accessible via the Human Protein Atlas portal linked just below the product name at the top of this page. The uniqueness and low cross-reactivity of the Prestige Antibodies® to other proteins are due to a thorough selection of antigen regions, affinity purification, and stringent selection. Prestige antigen controls are available for every corresponding Prestige Antibody and can be found in the linkage section.
Every Prestige Antibody is tested in the following ways:

• IHC tissue array of 44 normal human tissues and 20 of the most common cancer type tissues.
• Protein array of 364 human recombinant protein fragments.

Corresponding Antigen APREST71674,.
Physical form
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Prestige Antibodies is a registered trademark of Sigma-Aldrich Co. LLC
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

biological source	rabbit
Quality Level	100
antibody form	affinity isolated antibody
antibody product type	primary antibodies
clone	polyclonal
product line	Prestige Antibodies® Powered by Atlas Antibodies
form	buffered aqueous glycerol solution
species reactivity	human
packaging	antibody small pack of 25 µL
enhanced validation	recombinant expression
application(s)	immunoblotting: 0.04-0.4 µg/mL","immunohistochemistry: 1:200-1:500
immunogen sequence	REAYKTLSSLYSEMQKMNELENKAEPGTHLCIDVEDAMNITRKLWSRTFNCSVP
conjugate	unconjugated
UniProt accession no.	Q86WC4,
shipped in	wet ice
storage temp.	20°C
Gene Information	human ... OSTM1(28962)

Personal Protective Equipment	Eyeshields,, Gloves,, multi-purpose combination respirator cartridge (US),
RIDADR	NONH for all modes of transport
WGK Germany	WGK 1
Flash Point F	Not applicable
Flash Point C	Not applicable