BLOC1S3 Antibody

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.

NCBI Gene ID

388552

Swiss Prot

Q6QNY0

Accession Number

Q6QNY0

Host

Rabbit

Reactivity

Human

Immunogen

This 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.

Clonality

Polyclonal

Conjugation

Unconjugated

Type

Primary Antibodies

Field of Research

Signal Transduction

Purification

This antibody is purified through a protein A column, followed by peptide affinity purification.

Concentration

Batch dependent

Buffer

Supplied in PBS with 0.09% (W/V) sodium azide.

Modification

None

Shipping Conditions

Blue Ice

Storage Conditions

Store at 4˚ C for three months and -20˚ C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures.

Calculated Molecular Weight

21 kDa

Fragment

Rabbit Ig

Applications Notes

For WB starting dilution is: 1:1000

Symbol

BLOC1S3

NCBI Official Name

Biogenesis of lysosome-related organelles complex 1 subunit 3

NCBI Organism

Homo sapiens

Background Reference 01

Seshadri, S., et al. JAMA 303 (18) :1832-1840 (2010)

Background Reference 02

Lowe, J.K., et al. PLoS Genet. (2), E1000365 (2009) :

Background Reference 03

Olsen, J.V., et al. Cell 127 (3) :635-648 (2006)

Background Reference 04

Morgan, N.V., et al. Am. J. Hum. Genet. 78 (1) :160-166 (2006)

Other Product Names

Biogenesis of lysosome-related organelles complex 1 subunit 3, BLOC-1 subunit 3, BLOC1S3, BLOS3

Tested Applications

Protein ID

74749299

Physical Properties

Liquid

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