货号 |
bs-8506R-1 |
品牌 |
|
浓度 |
|
货期 |
现货 |
英文名称 |
Rabbit Anti-OSTM1 antibody |
中文名称 |
Rabbit Anti-OSTM1 antibody |
英文别名 |
GAIP-interacting protein N terminus; GIPN; GL antibody Grey lethal osteopetrosis; HSPC019; OPTB5; Osteopetrosis-associated transmembrane protein 1; Ostm1; OSTM1_HUMAN. |
反应物种(已验证) |
Human,Mouse,Rat |
反应物种(预测) |
Pig |
产品应用(已验证) |
WB,FCM |
产品应用(可推荐) |
IHC,IF,ELISA |
推荐稀释比例 |
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, |
克隆类型 |
多克隆 |
抗体来源 |
Rabbit |
理论分子量 |
33 |
细胞定位 |
细胞膜 |
性状 |
Liquid |
免疫原 |
KLH conjugated synthetic peptide derived from human OSTM1 |
抗原表位 |
21-120/334 |
抗原细胞定位 |
Extracellular |
亚型 |
IgG |
纯化方法 |
affinity purified by Protein A |
SUBCELLULAR |
Membrane; Single-pass type I membrane protein. |
Function |
Required for osteoclast and melanocyte maturation and function (By similarity). |
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 o |
SWISS |
Q86WC4 |
Gene ID |
28962 |
保存条件 |
Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. |
Important Note |
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
英文介绍 |
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. |