PLEKHM1 polyclonal, anti-human
€484.00
In stock
SKU
254173
Catalog Nr.: 254173
Size: 0.1 mg
Isotype: Rabbit Ig
Applications: E, WB, IHC
Datasheet
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Size: 0.1 mg
Isotype: Rabbit Ig
Applications: E, WB, IHC
Datasheet
Request Information
Protein Family: Enzymes
Pathway and Disease: Signaling Molecules and Interaction
Description:
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.
Alternate Names: PLEKHM1, Pleckstrin homolog domain containing family M member 1, OPTB6, B2, AP162
Application Notes: E: 1:500-1:1,000; WB: 1:100-1:500; IHC: 1:100-1:500
Accession No.: Q9Y4G2
Antigen: KLH-conjugated synthetic peptide encompassing a sequence within human PLEKHM1.
Format: Each vial contains 0.1 ml IgG in PBS pH 7.4 with 0.02% sodium azide. Antibody was purified by immunogen affinity chromatography.
Storage:
Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment.
Pathway and Disease: Signaling Molecules and Interaction
Description:
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.
Alternate Names: PLEKHM1, Pleckstrin homolog domain containing family M member 1, OPTB6, B2, AP162
Application Notes: E: 1:500-1:1,000; WB: 1:100-1:500; IHC: 1:100-1:500
Accession No.: Q9Y4G2
Antigen: KLH-conjugated synthetic peptide encompassing a sequence within human PLEKHM1.
Format: Each vial contains 0.1 ml IgG in PBS pH 7.4 with 0.02% sodium azide. Antibody was purified by immunogen affinity chromatography.
Storage:
Store at -20°C. Minimize freeze-thaw cycles. Product is guaranteed one year from the date of shipment.
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