Goat HPS3 Polyclonal Antibody | anti-HPS3 antibody

HPS3 (Cocoa, Hermansky-Pudlak Syndrome 3 Protein)

Cat. Num. AAA6004835

• Gene Names: HPS3; SUTAL
• Reactivity: Bovine, Human, Mouse, Porcine, Rat
• Applications: ELISA, Western Blot
• Purity: Affinity Purified; Purified by immunoaffinity chromatography.
• Synonyms: HPS3; Polyclonal Antibody; HPS3 (Cocoa; Hermansky-Pudlak Syndrome 3 Protein); Anti -HPS3 (Cocoa; anti-HPS3 antibody

• Host: Goat
• Reactivity: Bovine, Human, Mouse, Porcine, Rat
• Clonality: Polyclonal
• Specificity: Recognizes human HPS3. Species sequence homology: Bovine, mouse, porcine and rat.
• Purity/Purification: Affinity Purified; Purified by immunoaffinity chromatography.
• Form/Format: Supplied as a liquid in Tris saline, 0.02% sodium azide, pH 7.3, 0.5% BSA.

• Applicable Applications for anti-HPS3 antibody: ELISA (EL/EIA), Western Blot (WB)
• Application Notes: Suitable for use in ELISA and Western Blot.; Dilution: ELISA: 1:32,000; Western Blot: 1-3ug/ml, observed in A431 cell lysate on ~110kD bands
• Immunogen: Synthetic peptide corresponding to PYLLYCSRKKPLT, from human HPS3, at C-terminal (NP_115759.2).
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Chemiluminescence

((1ug/ml) staining of A431 lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.)

Related Product Information for anti-HPS3 antibody

Involved in early stages of melanosome biogenesis and maturation.

Product Categories/Family for anti-HPS3 antibody

Antibodies; Abs to Disease Markers

NCBI and Uniprot Product Information

• NCBI GI #: 19923642
• NCBI GeneID: 84343
• NCBI Accession #: NP_115759.2
• NCBI GenBank Nucleotide #: NM_032383.3
• UniProt Accession #: Q969F9; Q8WTV6; Q96AP1; Q96MR3; Q9H608; A8K6G6
• Molecular Weight: 113,736 Da
• NCBI Official Full Name: Hermansky-Pudlak syndrome 3 protein
• NCBI Official Synonym Full Names: Hermansky-Pudlak syndrome 3
• NCBI Official Symbol: HPS3
• NCBI Official Synonym Symbols: SUTAL
• NCBI Protein Information: Hermansky-Pudlak syndrome 3 protein
• UniProt Protein Name: Hermansky-Pudlak syndrome 3 protein
• Protein Family: Hermansky-Pudlak syndrome 3 protein
• UniProt Gene Name: HPS3
• UniProt Entry Name: HPS3_HUMAN

NCBI Description

This gene encodes a protein containing a potential clathrin-binding motif, consensus dileucine signals, and tyrosine-based sorting signals for targeting to vesicles of lysosomal lineage. The encoded protein may play a role in organelle biogenesis associated with melanosomes, platelet dense granules, and lysosomes. Mutations in this gene are associated with Hermansky-Pudlak syndrome type 3. Alternate splice variants exist, but their full length sequence has not been determined. [provided by RefSeq, Jul 2008]

Uniprot Description

HPS3: Involved in early stages of melanosome biogenesis and maturation. Defects in HPS3 are the cause of Hermansky-Pudlak syndrome type 3 (HPS3). 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. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Vesicle

Chromosomal Location of Human Ortholog: 3q24

Biological Process: pigmentation; organelle organization and biogenesis

Disease: Hermansky-pudlak Syndrome 3