PLEKHM1
Reactivité: Humain
IF (cc), IF (p)
Hôte: Lapin
Polyclonal
Cy3
Indications d'application
ELISA. Western blot: 1 - 2 μg/mL. Immunohistochemistry on paraffin sections. Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
Restrictions
For Research Use only
Concentration
1.0 mg/mL
Buffer
PBS containing 0.02 % sodium azide
Agent conservateur
Sodium azide
Précaution d'utilisation
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Conseil sur la manipulation
Avoid repeated freezing and thawing.
Stock
-20 °C
Stockage commentaire
Store the antibody (in aliquots) at -20 °C.
Antigène
PLEKHM1
(Pleckstrin Homology Domain Containing, Family M (With RUN Domain) Member 1 (PLEKHM1))
anticorps AP162, anticorps B2, anticorps OPTB6, anticorps BC038943, anticorps D330036J23Rik, anticorps pleckstrin homology and RUN domain containing M1, anticorps pleckstrin homology domain containing, family M (with RUN domain) member 1, anticorps PLEKHM1, anticorps Plekhm1
Sujet
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.Synonyms: KIAA0356, PH domain-containing family M member 1, Pleckstrin homology domain-containing family M member 1