Rabbit anti-Human HPS5 Polyclonal Antibody | anti-HPS5 antibody

HPS5, NT (HPS5, AIBP63, KIAA1017, Hermansky-Pudlak syndrome 5 protein, Alpha-integrin-binding protein 63, Ruby-eye protein 2 homolog) (PE)

Cat. Num. AAA6308275

• Gene Names: HPS5; AIBP63
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: HPS5; Polyclonal Antibody; NT (HPS5; AIBP63; KIAA1017; Hermansky-Pudlak syndrome 5 protein; Alpha-integrin-binding protein 63; Ruby-eye protein 2 homolog) (PE); Ruby-eye protein 2 homolog; anti-HPS5 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with R-Phycoerythrin (PE).

• Applicable Applications for anti-HPS5 antibody: Western Blot (WB), FLISA
• Application Notes: WB: 1:100-500; FLISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: HPS5 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 88-116 amino acids from the N-terminal region of human HPS5.
• Conjugate: PE
• Note: Preservative Free
• Special Handling: Light sensitive
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: PE conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(HPS5 Antibody (N-term) western blot analysis in NCI-H292 cell line lysates (35ug/lane).This demonstrates the HPS5 antibody detected the HPS5 protein (arrow).)

Related Product Information for anti-HPS5 antibody

This gene encodes a protein that may play a role in organelle biogenesis associated with melanosomes, platelet dense granules, and lysosomes. This protein interacts with Hermansky-Pudlak syndrome 6 protein and may interact with the cytoplasmic domain of integrin, alpha-3. Mutations in this gene are associated with Hermansky-Pudlak syndrome type 5. Multiple transcript variants encoding two distinct isoforms have been identified for this gene.

Product Categories/Family for anti-HPS5 antibody

Antibodies; Integrin

References

Helip-Wooley, A., et al. J. Invest. Dermatol. 127(6):1471-1478(2007). Matsuoka, S., et al. Science 316(5828):1160-1166(2007). Huizing, M., et al. Platelets 18(2):150-157(2007). Huizing, M., et al. Traffic 5(9):711-722(2004). Di Pietro, S.M., et al. Traffic 5(4):276-283(2004).

NCBI and Uniprot Product Information

• NCBI GI #: 31657127
• NCBI GeneID: 11234
• NCBI Accession #: NP_009147.3
• NCBI GenBank Nucleotide #: NM_007216.3
• UniProt Accession #: Q9UPZ3; O95942; Q8N4U0; A8K6J8; A8K8S1; D3DQX9; D3DQY0
• Molecular Weight: 127,449 Da
• NCBI Official Full Name: Hermansky-Pudlak syndrome 5 protein isoform b
• NCBI Official Synonym Full Names: Hermansky-Pudlak syndrome 5
• NCBI Official Symbol: HPS5
• NCBI Official Synonym Symbols: AIBP63
• NCBI Protein Information: Hermansky-Pudlak syndrome 5 protein; ruby-eye protein 2 homolog; alpha integrin binding protein 63; alpha-integrin-binding protein 63
• UniProt Protein Name: Hermansky-Pudlak syndrome 5 protein
• Protein Family: Hermansky-Pudlak syndrome 5 protein
• UniProt Gene Name: HPS5
• UniProt Synonym Gene Names: AIBP63; KIAA1017; Ru2
• UniProt Entry Name: HPS5_HUMAN

NCBI Description

This gene encodes a protein that may play a role in organelle biogenesis associated with melanosomes, platelet dense granules, and lysosomes. This protein interacts with Hermansky-Pudlak syndrome 6 protein and may interact with the cytoplasmic domain of integrin, alpha-3. Mutations in this gene are associated with Hermansky-Pudlak syndrome type 5. Multiple transcript variants encoding two distinct isoforms have been identified for this gene. [provided by RefSeq, Jul 2008]

Uniprot Description

HPS5: May regulate the synthesis and function of lysosomes and of highly specialized organelles, such as melanosomes and platelet dense granules. Regulates intracellular vesicular trafficking in fibroblasts. May be involved in the regulation of general functions of integrins. Defects in HPS5 are the cause of Hermansky-Pudlak syndrome type 5 (HPS5). 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. Belongs to the HPS5 family. 2 isoforms of the human protein are produced by alternative splicing.

Chromosomal Location of Human Ortholog: 11p14

Biological Process: pigmentation; organelle organization and biogenesis; blood coagulation

Disease: Hermansky-pudlak Syndrome 5