BL1S3 rabbit pAb
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. [provided by RefSeq, Jul 2008],
Product Specifications
Background
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. [provided by RefSeq, Jul 2008]
UniProt
Q6QNY0
Swiss Prot
Q6QNY0
Reactivity
Human; Mouse
Immunogen
Synthesized peptide derived from human BL1S3 AA range: 142-192
Target
BL1S3
Clonality
Polyclonal
Source
Rabbit
Applications
WB
Concentration
1 mg/ml
Dilution
WB 1:500-2000
Buffer
-20°C/1 year
Storage Conditions
-20°C/1 year
Fragment
IgG
Subcellular Location
Cytoplasm .
Gene ID (Human)
388552
Available Sizes
Frequently Asked Questions
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