product summary
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company name :
MyBioSource
product type :
antibody
product name :
Rabbit anti-human Hermansky-Pudlak syndrome 1 polyclonal Antibody
catalog :
MBS716585
quantity :
0.05 mL
price :
375 USD
clonality :
polyclonal
host :
rabbit
conjugate :
nonconjugated
reactivity :
human
application :
western blot, ELISA, enzyme immunoassay
more info or order :
MyBioSource product webpage
product information
catalog number :
MBS716585
products type :
Antibody
products full name :
Rabbit anti-human Hermansky-Pudlak syndrome 1 polyclonal Antibody
products short name :
Hermansky-Pudlak syndrome 1
products name syn :
Hermansky-Pudlak syndrome 1; HPS1; HPS; MGC5277
other names :
Hermansky-Pudlak syndrome 1; Hermansky-Pudlak syndrome 1 protein; Hermansky-Pudlak syndrome 1 protein; Hermansky-Pudlak syndrome 1
other gene names :
HPS1; HPS1; HPS; HPS
uniprot entry name :
HPS1_HUMAN
clonality :
Polyclonal
isotype :
IgG
host :
Rabbit
reactivity :
Human?Other species are not tested. Please decide the specificity by homology.
sequence length :
324
purity :
Antigen Affinity Purified
form :
Liquid
storage stability :
Shipped at 4 degree C Upon delivery aliquot and store at -20 degree C or -80 degree C. Avoid repeated freeze.
tested application :
ELISA (EIA), Western Blot (WB)
app notes :
ELISA: Use at an assay dependent dilution. WB : 1:200-1:2000 (Recommender dilutions). Positive WB detected in: L02 cells, HEK-293 cells, K-562 cells, Raji cells, Transfected HEK-293 cells, Y79 cells
other info1 :
Immunogen: Human HPS1. Conjugate : Non-conjugated
other info2 :
Storage Buffer: PBS with 0.02% Sodium Azide, 50% Glycerol, pH 7.3.
products description :
Component of multiple cytoplasmic organelles. Apparently crucial for their normal development and function. May be involved in intracellular protein sorting.
ncbi gi num :
12652845
ncbi acc num :
AAH00175.1
ncbi summary :
This gene encodes a protein that may play a role in organelle biogenesis associated with melanosomes, platelet dense granules, and lysosomes. The encoded protein is a component of three different protein complexes termed biogenesis of lysosome-related organelles complex (BLOC)-3, BLOC4, and BLOC5. Mutations in this gene are associated with Hermansky-Pudlak syndrome type 1. Multiple transcript variants encoding distinct isoforms have been identified for this gene; the full-length sequences of some of these have not been determined yet. [provided by RefSeq, Jul 2008]
uniprot summary :
HPS1: Component of multiple cytoplasmic organelles. Apparently crucial for their normal development and function. May be involved in intracellular protein sorting. Defects in HPS1 are the cause of Hermansky-Pudlak syndrome type 1 (HPS1). Hermansky-Pudlak syndrome (HPS) is a genetically heterogeneous, rare, autosomal recessive disorder characterized by oculocutaneous albinism, bleeding due to platelet storage pool deficiency, and lysosomal storage defects. This syndrome results from defects of diverse cytoplasmic organelles including melanosomes, platelet dense granules and lysosomes. Ceroid storage in the lungs is associated with pulmonary fibrosis, a common cause of premature death in individuals with HPS. 4 isoforms of the human protein are produced by alternative splicing. Protein type: Unknown function. Chromosomal Location of Human Ortholog: 10q23.1-q23.3. Cellular Component: lysosome; integral to plasma membrane; cytoplasmic membrane-bound vesicle; cytoplasm. Molecular Function: protein dimerization activity; protein binding. Biological Process: secretion of lysosomal enzymes; visual perception; retina development in camera-type eye; lysosome organization and biogenesis; melanocyte differentiation; blood coagulation; positive regulation of natural killer cell activation; eye pigmentation. Disease: Hermansky-pudlak Syndrome 1
size1 :
0.05 mL
price1 :
375 USD
size2 :
0.15 mL
price2 :
715
more info or order :
MyBioSource product webpage
company information
MyBioSource
P.O. Box 153308
San Diego, CA 92195-3308
sales@mybiosource.com
https://www.mybiosource.com
1-888-627-0165
headquarters: USA
MyBioSource, LLC was orginally founded in Vancouver by three enthusiastic scientists who are passionate about providing the world with the best reagents available. Together, they form a company with a big vision known as MyBioSource. MyBioSource is now located in San Diego, California, USA.

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