HPA010851
Anti-OSTM1 antibody produced in rabbit
Prestige Antibodies® Powered by Atlas Antibodies, affinity isolated antibody, buffered aqueous glycerol solution
Sinonimo/i:
Anti-Osteopetrosis-associated transmembrane protein 1 precursor antibody produced in rabbit
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About This Item
Prodotti consigliati
Origine biologica
rabbit
Coniugato
unconjugated
Forma dell’anticorpo
affinity isolated antibody
Tipo di anticorpo
primary antibodies
Clone
polyclonal
Nome Commerciale
Prestige Antibodies® Powered by Atlas Antibodies
Forma fisica
buffered aqueous glycerol solution
Reattività contro le specie
human
Convalida avanzata
recombinant expression
Learn more about Antibody Enhanced Validation
tecniche
immunoblotting: 0.04-0.4 μg/mL
immunohistochemistry: 1:200-1:500
Sequenza immunogenica
REAYKTLSSLYSEMQKMNELENKAEPGTHLCIDVEDAMNITRKLWSRTFNCSVP
N° accesso UniProt
Condizioni di spedizione
wet ice
Temperatura di conservazione
−20°C
modifica post-traduzionali bersaglio
unmodified
Informazioni sul gene
human ... OSTM1(28962)
Descrizione generale
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.
Immunogeno
Osteopetrosis-associated transmembrane protein 1 precursor recombinant protein epitope signature tag (PrEST)
Applicazioni
Anti-OSTM1 antibody produced in rabbit, a Prestige Antibody, is developed and validated by the Human Protein Atlas (HPA) project . 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.
Azioni biochim/fisiol
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.
Caratteristiche e vantaggi
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:
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.
Linkage
Corresponding Antigen APREST71674
Stato fisico
Solution in phosphate-buffered saline, pH 7.2, containing 40% glycerol and 0.02% sodium azide
Note legali
Prestige Antibodies is a registered trademark of Merck KGaA, Darmstadt, Germany
Esclusione di responsabilità
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.
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Codice della classe di stoccaggio
10 - Combustible liquids
Classe di pericolosità dell'acqua (WGK)
WGK 1
Punto d’infiammabilità (°F)
Not applicable
Punto d’infiammabilità (°C)
Not applicable
Dispositivi di protezione individuale
Eyeshields, Gloves, multi-purpose combination respirator cartridge (US)
Certificati d'analisi (COA)
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I documenti relativi ai prodotti acquistati recentemente sono disponibili nell’Archivio dei documenti.
Claus-Eric Ott et al.
Bone, 55(2), 292-297 (2013-05-21)
Autosomal recessive osteopetrosis (ARO, MIM 259700) is a genetically heterogeneous rare skeletal disorder characterized by failure of osteoclast resorption leading to pathologically increased bone density, bone marrow failure, and fractures. In the neuronopathic form neurological complications are especially severe and
Philipp F Lange et al.
Nature, 440(7081), 220-223 (2006-03-10)
Mutations in ClC-7, a late endosomal/lysosomal member of the CLC family of chloride channels and transporters, cause osteopetrosis and lysosomal storage disease in humans and mice. Severe osteopetrosis is also observed with mutations in the OSTM1 gene, which encodes a
Nader Chalhoub et al.
Nature medicine, 9(4), 399-406 (2003-03-11)
The spontaneous mouse grey-lethal (gl) mutation is responsible for a coat color defect and for the development of the most severe autosomal recessive form of osteopetrosis. Using a positional cloning approach, we have mapped and isolated the gl locus from
Alessandra Pangrazio et al.
Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research, 21(7), 1098-1105 (2006-07-04)
We report three novel osteopetrosis patients with OSTM1 mutations and review two that have been previously described. Our analysis suggests that OSTM1 defines a new subset of patients with severe central nervous system involvement. This defect is also present in
Bruno Maranda et al.
Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research, 23(2), 296-300 (2007-10-10)
Infantile ARO is a genetic disorder characterized by osteoclast dysfunction that leads to osteopetrosis. We describe a novel mutation affecting the OSTM1 locus responsible for ARO. In addition to common clinical features of osteopetrosis, the patient developed a unique neuronal
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