Product Name	OSTM1
Chinese Name	骨硬化病相关Transmembrane protein1抗体
Alias	GAIP-interacting protein N terminus; GIPN; GL antibody Grey lethal osteopetrosis; HSPC019; OPTB5; Osteopetrosis-associated transmembrane protein 1; Ostm1; OSTM1_HUMAN.
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Human, Mouse, Rat,  (predicted: Pig, )
Applications	WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 Flow-Cyt=1μg/Test IF=1:50-200 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	33kDa
Cellular localization	The cell membrane
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human OSTM1: 21-120/334 <Extracellular>
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	OSTM1 (osteopetrosis associated transmembrane protein 1), also known as gl (gray-lethal) or HSPC019, is a 338 amino acid single-pass type I membrane protein that is expressed primarily in osteoclasts and melanocytes as well as brain, kidney and spleen. Bone autosomal recessive osteopetrosis (ARO) is the most severe form of hereditary bone disease whose cellular basis is in the osteoclast and is characterized by abnormally dense bone, due to defective resorption of immature bone. ARO is suggested to be caused by mutations in the OSTM1 gene. The disorder 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. Defects in the OSTM1 gene are also the cause of the spontaneous gl mutant, which is responsible for a coat color defect in mice. Function: Required for osteoclast and melanocyte maturation and function (By similarity). Subcellular Location: Membrane; Single-pass type I membrane protein. DISEASE: Defects in OSTM1 are the cause of osteopetrosis autosomal recessive type 5 (OPTB5) [MIM:259720]; also called infantile malignant osteopetrosis 3. Osteopetrosis is a rare genetic disease characterized by abnormally dense bone, due to defective resorption of immature bone. The disorder 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. 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. SWISS: Q86WC4 Gene ID: 28962 Database links: Entrez Gene: 28962 Human Entrez Gene: 14628 Mouse Entrez Gene: 499474 Rat Omim: 607649 Human SwissProt: Q86WC4 Human SwissProt: Q8BGT0 Mouse Unigene: 226780 Human
Product Picture	Sample: Lane 1: Mouse Spleen tissue lysates Lane 2: Mouse Lung tissue lysates Lane 3: Rat Liver tissue lysates Lane 4: Rat Spleen tissue lysates Lane 5: Human U87MG cell lysates Lane 6: Human U251 cell lysates Lane 7: Human THP-1 cell lysates Primary: Anti-OSTM1 (SL8506R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 33 kD Observed band size: 35 kD Positive control: mouse Splenocytes(2% Paraformaldehyde-fixed ) Isotype Control Antibody: Rabbit IgG; Dilution: 1μg in 100 μl 1 X PBS containing 0.5% BSA Secondary Antibody: Goat anti-rabbit IgG-FITC; Dilution: 1:200 in 1 X PBS containing 0.5% BSA Primary Antibody :rabbit Anti-OSTM1 (SL8506R); Dilution: 1μg in 100 μl 1X PBS containing 0.5% BSA

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