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Mouse Anti-OSTM1 (AA 183-282) Recombinant Antibody (4H1) (CBMAB-O0656-CQ)

This product is a mouse antibody that recognizes OSTM1 (AA 183-282). The antibody 4H1 can be used for immunoassay techniques such as: ELISA, WB.
See all OSTM1 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
4H1
Antibody Isotype
IgG2a, Κ
Application
ELISA, WB

Basic Information

Immunogen
OSTM1 (NP_054747.2, 183aa-282aa) partial Recombinant protein with GST tag
Specificity
Human
Antibody Isotype
IgG2a, Κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, pH 7.4
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.
Epitope
AA 183-282

Target

Full Name
osteopetrosis associated transmembrane protein 1
Introduction
This gene encodes a protein that may be involved in the degradation of G proteins via the ubiquitin-dependent proteasome pathway. The encoded protein binds to members of subfamily A of the regulator of the G-protein signaling (RGS) family through an N-terminal leucine-rich region. This protein also has a central RING finger-like domain and E3 ubiquitin ligase activity. This protein is highly conserved from flies to humans. Defects in this gene may cause the autosomal recessive, infantile malignant form of osteopetrosis.
Entrez Gene ID
28962
UniProt ID
Q86WC4
Alternative Names
Osteopetrosis Associated Transmembrane Protein 1; Chloride Channel 7 Beta Subunit; CLCN7 Accessory Beta Subunit; GL; Osteopetrosis-Associated Transmembrane Protein 1; GAIP-Interacting Protein N Terminus
Function
Required for osteoclast and melanocyte maturation and function.
Biological Process
Osteoclast differentiationManual Assertion Based On ExperimentIBA:GO_Central
Transepithelial chloride transportManual Assertion Based On ExperimentIDA:ComplexPortal
Cellular Location
Lysosome membrane
Requires CLCN7 to travel to lysosomes.
Involvement in disease
Osteopetrosis, autosomal recessive 5 (OPTB5):
A rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. Osteopetrosis occurs in two forms: a severe autosomal recessive form occurring in utero, infancy, or childhood, and a benign autosomal dominant form occurring in adolescence or adulthood. Recessive osteopetrosis commonly manifests in early infancy with macrocephaly, feeding difficulties, evolving blindness and deafness, bone marrow failure, severe anemia, and hepatosplenomegaly. Deafness and blindness are generally thought to represent effects of pressure on nerves. OPTB5 patients manifest primary central nervous system involvement in addition to the classical stigmata of severe bone sclerosis, growth failure, anemia, thrombocytopenia and visual impairment with optic atrophy.
Topology
Lumenal: 32-284
Helical: 285-305
Cytoplasmic: 306-334
PTM
Undergoes proteolytic cleavage in the luminal domain, the cleaved fragments might be linked by disulfide bonds with the remnant of the protein.
Highly N-glycosylated.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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