PLEKHM1 Antilichaam

PLEKHM1 Antibody: PLEKHM1 is a member of the M family of Pleckstrin homolog domain-containing proteins, a group of proteins containing a RUN domain, two pleckstrin homology domains, and a cysteine-rich domain. It was identified through segregation analysis as a cause of osteopetrosis in humans. PLEKHM1 co-localizes with Rab7 to late endosomal/lysosomal vesicles in HEK293 and osteoclast-like cells, with this co-localization dependent on the prenylation of Rab7. Monocytes from a patient homozygous for a mutated form of PLEKHM1differentiated into osteoclasts normally, but failed to form ruffled borders and showed little evidence of bone resorbtion when cultured on dentine discs. Another mutation of PLEKHM1 impaired vesicular acidification and increased TRACP secretion in osteoclasts, suggesting that PLEKHM1 has critical roles in endosomal maturation and may be important in osteoclast-osteoblast cross-talk.

9842

Q9Y4G2

Q9Y4G2

Human

Unconjugated

Primary Antibodies

Stem Cell

PLEKHM1 Antibody is affinity chromatography purified via peptide column.

Cat. No. 1302 - Human Lung Tissue Lysate

1 mg/mL

Predicted species reactivity based on immunogen sequence: Rat: (89%), Mouse: (83%)

PLEKHM1 Antibody is supplied in PBS containing 0.02% sodium azide.

None

Blue Ice

PLEKHM1 antibody can be stored at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

IgG

PLEKHM1

Cat. No. 10-101 - Human Lung Tissue Slide

Pleckstrin homology domain containing, family M (with RUN domain) member 1

Homo sapiens

Van Wesenbeeck L, Odgren PR, Mackay CA, et al. Localization of the gene causing the osteopetrotic phenotype in the incisors absent (ia) rat on chromosome 10q32.1. J. Bone Miner. Res.2004; 19:183-9.

Van Wesenbeeck L, Odgren PR, Coxon FP, et al. Involvement of PLEKHM1 in osteoclastic vesicular transport and osteopetrosis in incisors absent rats and humans. J. Clin. Invest.2007; 117:919-30.

Del Fattore A, Fornari R, Van Wesenbeeck L, et al. A new heterozygous mutation (R714C) of the osteopetrosis gene, pleckstrin homolog domain containing family M (with run domain) member 1 (PLEKHM1), impairs vesicular acidification and increases TRACP secretion in osteoclasts. J. Bone Miner. Res.2008; 23:380-91.

PLEKHM1 Antibody: B2, AP162, OPTB6, KIAA0356, Pleckstrin homology domain-containing family M member 1, 162 kDa adapter protein, PH domain-containing family M member 1

ELISA, WB, IHC-P, IF

160419247

Liquid