PLEKHM1 Antibody

Catalog Number:

25003

Amount:

100μg

Price:

$319

Swiss-Prot No:

Q9Y4G2

Form of Antibody:

Supplied in PBS containing 0.02% sodium azide.

Storage/Stability:

Immunogen:

Raised against an 18 amino acid peptide from near the amino terminus of human PLEKHM1.

Purification:

Affinity chromatography purified via peptide column

Specificity/Sensitivity:

Applications:

ELISA WB IHC

Background:

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.

References:

appl_detail:

Datasheet PDF