Hermansky-Pudlak Syndrome 3 (HPS3) (C-Term) antibody

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Antigen
Synonyms SUTAL, HPS3
Epitope
C-Term
(2), (2), (1), (1), (1)
Reactivity
Human
(15), (5), (3)
Host
Goat
(11), (5), (1)
Clonality
Polyclonal
Application
Western Blotting (WB)
(16), (6), (5), (1), (1), (1)
Pubmed 2 references available
Catalog no. ABIN374450
Quantity 0.1 mg
Price
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Immunogen Peptide from the C Terminus of the protein sequence according to NP_115759
Sequence PYLLYCSRKKPLT
Specificity This antibody recognizes HPS3/Cocoa Species Reactivity: Tested: Human.
Purification Ammonium Sulphate Precipitation followed by antigen Affinity Chromatography using the immunizing peptide.
Alternative Name HPS3
Background HPS3 is involved in early stages of melanosome biogenesis and maturation. Defects in HPS3 are the cause of the cocoa (coa) mutant, and of Hermansky-Pudlak syndrome type 3 (HPS3). HPS3 is an 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.
Alternate names: Hermansky-Pudlak syndrome 3
Gene ID 84343
NCBI Accession NP_115759.2
UniProt Q969F9
Research Area Signaling
Application Notes Peptide ELISA: > 1/32,000. Western Blot: 1-3 µg/ml. Detects a band of Approx 110kDa in A431 cell lysate(Predicted Molecular Weight: 114 kDa). Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
Restrictions For Research Use only
Format Liquid
Concentration 0.5 mg/mL
Buffer Tris buffered saline, pH 7.3 containing 0.02% Sodium Azide as preservative and 0.5% BSA as stabilizer.
Preservative Sodium azide
Handling Advice Avoid repeated freezing and thawing.
Storage -20 °C
Storage Comment Store the antibody (in aliquots) at -20°C.
Expiry Date 12 months
Supplier Images
anti-Hermansky-Pudlak Syndrome 3 (HPS3) (C-Term) antibody anti-Hermansky-Pudlak Syndrome 3 (HPS3) (C-Term) antibody
Background publications Gautam, Chintala, Li et al.: "The Hermansky-Pudlak syndrome 3 (cocoa) protein is a component of the biogenesis of lysosome-related organelles complex-2 (BLOC-2)." in: The Journal of biological chemistry, Vol. 279, Issue 13, pp. 12935-42, 2004 (PubMed).

Smeazzetto, Schröder, Thiel et al.: "Phospholamban generates cation selective ion channels." in: Physical chemistry chemical physics : PCCP, Vol. 13, Issue 28, pp. 12935-9, 2011 (PubMed).

Hosts (11), (5), (1)
Reactivities (15), (5), (3)
Applications (16), (6), (5), (1), (1), (1)
Epitopes (2), (2), (1), (1), (1)
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