HPS3 Antibody

Western Blot: HPS3 Antibody [NB100-1445] - (1ug/ml) staining of A431 lysate (35ug protein in RIPA buffer). Primary incubation was 1 hour. Detected by chemiluminescence.

• Reactivity: Hu, Mu, Rt, Po, Bv
• Applications: WB, ELISA
• Clonality: Polyclonal
• Host: Goat
• Conjugate: Unconjugated
• Concentration: 0.5 mg/ml

HPS3 Antibody Summary

• Immunogen: Peptide with sequence PYLLYCSRKKPLT corresponding to C-Terminus according to NP_115759.2.
• Localization: Cytoplasmic
• Predicted Species: Mouse (100%), Rat (100%), Porcine (100%), Bovine (100%). Backed by our 100% Guarantee.
• Isotype: IgG
• Clonality: Polyclonal
• Host: Goat
• Gene: HPS3
• Purity: Immunogen affinity purified

Applications/Dilutions

• Dilutions:
  • Peptide ELISA Detection limit 1:32000
  • Western Blot 1 - 3 ug/ml
• Application Notes: WB: Approx. 110 kDa band observed in A431 lysate (calculated MW of 114 kDa band according to NP_115759).
• Control:

  HPS3 Overexpression Lysate
  A-431 Whole Cell Lysate

Packaging, Storage & Formulations

• Storage: Store at -20C. Avoid freeze-thaw cycles.
• Buffer: Tris saline (20 mM Tris pH 7.3, 150 mM NaCl), 0.5% BSA
• Preservative: 0.02% Sodium Azide
• Concentration: 0.5 mg/ml
• Purity: Immunogen affinity purified

Alternate Names for HPS3 Antibody

• DKFZp686F0413
• FLJ22704
• Hermansky-Pudlak syndrome 3 protein
• Hermansky-Pudlak syndrome 3
• SUTAL

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.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Publications for HPS3 Antibody (NB100-1445)(1)

Publication using NB100-1445

• Gautam R, Chintala S, Li W et al. The Hermansky-Pudlak syndrome 3 (cocoa) protein is a component of the biogenesis of lysosome-related organelles complex-2 (BLOC-2). J Biol Chem 2004-03-26 [PMID: 14718540]

Bioinformatics

• Gene Symbol: HPS3
• Entrez:
  • 84343(Human)
• Uniprot:
  • NP_115759.(Human)