Anti-HPS1 Antibody

$99.00$319.00

Reactivity: Human
Applications: ELISA,IHC
Conjugation: Various
Supplier: CUSABIO BIOTECH CO.

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Rabbit Anti-HPS1 Antibody (CSB-PA856438ESR1HU)

Supplier: CUSABIO BIOTECH CO.

Alternative names:

Hermansky-Pudlak syndrome 1 protein Antibody,HPS1 Antibody,HPS Antibody

More alternative names for the antibody
Hermansky Pudlak syndrome 1 protein antibody|Hermansky Pudlak syndrome gene antibody|Hermansky Pudlak syndrome type 1 antibody|Hermansky-Pudlak syndrome 1 antibody|Hermansky-Pudlak syndrome 1 protein antibody|HPS antibody|HPS1 antibody|HPS1_HUMAN antibody|MGC5277 antibody
Anti-HPS1 antibody [EP8429(2)] (ab174301)
Close sc-101435|sc-33374|

Recommended applications: ELISA, IHC

Recommended dilution: Recommended dilution:IHC:1:20-1:200

Recommended protocols: check protocols

Name

Anti-HPS1 Antibody

Catalogue No.

CSB-PA856438ESR1HU

Reactivity

Human

Immunogen

Recombinant human Hermansky-Pudlak syndrome 1 protein (1-324AA)

Host

Rabbit

Applications

ELISA, IHC

Recommended dilution

Recommended dilution:IHC:1:20-1:200

Clonality

Polyclonal

Conjugation

unconjugated

Isotype

IgG

Molecular weight

79 kDa

Purification

Antigen Affinity Purified

Storage

Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.

Database links

Entrez Gene:3257( Human), Entrez Gene:192236( Mouse), Entrez Gene:114638( Rat), Omim:604982( Human), SwissProt:Q92902( Human), SwissProt:O08983( Mouse), Unigene:404568( Human), Unigene:218381( Mouse), Unigene:17691( Rat)

Protein function

Component of the BLOC-3 complex, a complex that acts as a guanine exchange factor (GEF) for RAB32 and RAB38, promotes the exchange of GDP to GTP, converting them from an inactive GDP-bound form into an active GTP-bound form. The BLOC-3 complex plays an important role in the control of melanin production and melanosome biogenesis and promotes the membrane localization of RAB32 and RAB38 (PubMed:23084991). .

Protein tissue specificity

Ubiquitous.

Involvement in disease

Hermansky-Pudlak syndrome 1 (HPS1) [MIM:203300]: A form of Hermansky-Pudlak syndrome, a genetically heterogeneous 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. . Note=The disease is caused by mutations affecting the gene represented in this entry.

Research area

All research areas>Signaling Intermediates>HPS
(View all antibody categories related to Signaling Intermediates)

Note

AntibodyPlus provides antibody trial sample for your own antibody validation and collects antibody reviews.

Supplier

CUSABIO BIOTECH CO.

Product type

Primary antibody

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