石骨症相关蛋白PLEKHM1抗体

Quantity size:

0.2ml

Concentration:

1mg/ml 石骨症相关蛋白PLEKHM1抗体  Buffer = 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Background:

Involved in vesicular transport in the osteoclast (By similarity). May have a role in sialyl-lex-mediated transduction of apoptotic signals.
Tissue specificity: Expressed in placenta, liver, prostate, thymus, spleen, ovary, colon, colon carcinoma and peripheral blood lymphocytes (PBL). Weakly expressed in brain, lung, kidney, and testis. 石骨症相关蛋白PLEKHM1抗体No expression in heart, skeletal muscle, pancreas and small intestine. Predominantly expressed in the breast carcinoma cell line MCF-7.
Involvement in disease:Defects in PLEKHM1 are the cause of osteopetrosis autosomal recessive type 6 (OPTB6); also known as autosomal recessive osteopetrosis intermediate form. Osteopetrosis is a rare genetic disease characterized by abnormally dense石骨症相关蛋白PLEKHM1抗体 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. Autosomal recessive osteopetrosis is usually associated with normal or elevated amount of non-functional osteoclasts.

石骨症相关蛋白PLEKHM1抗体Also known as:

162 kDa adapter protein; AP162; PH domain-containing family M member 1; PKHM1_HUMAN; Pleckstrin homology domain-containing family M member 1; PLEKHM1.

Specificity:

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Rabbit Polyclonal IgG, affinity purified by Protein A.

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Reacts with: Human, Mouse, Rat, Pig, Horse, Rabbit, .

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Immunogen: KLH conjugated synthetic peptide derived from human PLEKHM1.

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石骨症相关蛋白PLEKHM1抗体Predicted Molecular Weight: 117kDa.

Storage:

Shipped at 4℃, Store at -20℃ (Avoid repeated freeze/thaw cycles).

Application:

WB=1:100-500  ELISA=1:500-1000  IHC-P=1:100-500  IHC-F=1:100-500  IF=1:100-500   
Not yet tested in other applications. 
Optimal working dilutions must be determined by the end user.

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