Technical Data
032534
BLOC1S3, CT (BLOC1S3, BLOS3, Biogenesis of lysosome-related organelles complex 1 subunit 3)
Description:
This gene encodes a protein that is a component of the BLOC1 multi-subunit protein complex. This complex is necessary for the biogenesis of specialized organelles of the endosomal-lysosomal system, including platelet dense granules and melanosomes. Mutations in this gene cause Hermansky-Pudlak syndrome 8, a disease characterized by lysosomal storage defects, bleeding due to platelet storage pool deficiency, and oculocutaneous albinism.

Applications:
Suitable for use in Western Blot, ELISA

Recommended Dilution:
ELISA: 1:1,000
Western Blot: 1:100-500

Storage and Stability:
May be stored at 4C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20C. Aliquots are stable for 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
TypeIsotypeCloneGrade
PabIgGAffinity Purified
SizeStorageShippingSourceHost
200ul-20CBlue IceHumanRabbit
Concentration:
As reported
Immunogen:
BLOC1S3 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 176-202 amino acids from the C-terminal region of human BLOC1S3.
Purity:
Purified by Protein A affinity chromatography.
Form
Supplied as a liquid in PBS, pH 7.2, 0.09% sodium azide.
Specificity:
Human
Intended for research use only. Not for use in human, therapeutic, or diagnostic applications.
Seshadri, S., et al. JAMA 303(18):1832-1840(2010)
Lowe, J.K., et al. PLoS Genet. 5 (2), E1000365 (2009) :
Olsen, J.V., et al. Cell 127(3):635-648(2006)
Morgan, N.V., et al. Am. J. Hum. Genet. 78(1):160-166(2006)
Starcevic, M., et al. J. Biol. Chem. 279(27):28393-28401(2004)