HPS3 Recombinant Protein Antigen

• Reactivity: Hu
• Applications: AC

HPS3 Recombinant Protein Antigen Summary

• Description: A recombinant protein antigen with a N-terminal His6-ABP tag corresponding to human HPS3.

  Source: E. coli

  Amino Acid Sequence: YVNWRNKRTENSRVCIRMIGHNVEGPFSKAFRDQMYIIEMPLSEAPLCISCCPVKGDLLVGCTNKLVLFSLKYQIINEEFSLLDFER

  Fusion Tag: N-terminal His6ABP (ABP = Albumin Binding Protein derived from Streptococcal Protein G)

  This product is intended to be used as a blocking antigen for antibody competition assays. Any other use of this antigen is done at the risk of the user. The use of this product for commercial production is strictly prohibited. Please contact technical support if you have any questions.

• Source: E. coli
• Protein/Peptide Type: Recombinant Protein Antigen
• Gene: HPS3
• Purity: >80% by SDS-PAGE and Coomassie blue staining

Applications/Dilutions

• Dilutions:
  • Antibody Competition 10 - 100 molar excess
• Application Notes: This recombinant antigen is only intended to be used as a blocking agent to confirm antibody specificity with the corresponding antibody, catalog number NBP2-57010.

  It is purified by IMAC chromatography, and the expected concentration is greater than 0.5 mg/ml.

  For current lot information, including availability, please contact our technical support team click nb-technical@bio-techne.com

• Theoretical MW: 28 kDa.
  Disclaimer note: The observed molecular weight of the protein may vary from the listed predicted molecular weight due to post translational modifications, post translation cleavages, relative charges, and other experimental factors.

Packaging, Storage & Formulations

• Storage: Store at -20C. Avoid freeze-thaw cycles.
• Buffer: PBS and 1M Urea, pH 7.4.
• Preservative: No Preservative
• Purity: >80% by SDS-PAGE and Coomassie blue staining

Alternate Names for HPS3 Recombinant Protein Antigen

• 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. Peptides and proteins are guaranteed for 3 months from date of receipt.

Bioinformatics

• Gene Symbol: HPS3