技术文章

Recombinant Human HPS3 Protein: Function & Research Use

Recombinant Human HPS3 Protein is a key research tool for investigating the molecular mechanisms of lysosome-related organelle biogenesis and the pathophysiology of Hermansky-Pudlak syndrome type 3.

Gene Name
HPS3
Species
Human (Homo sapiens)
Protein Full Name
Hermansky-Pudlak Syndrome 3 Protein
Complex Association
BLOC-2 (Biogenesis of Lysosome-related Organelles Complex-2)
Primary Function
Regulation of lysosome-related organelle biogenesis and intracellular vesicle trafficking
Disease Relevance
Mutations cause Hermansky-Pudlak Syndrome type 3 (HPS-3), characterized by oculocutaneous albinism and platelet storage pool deficiency
SKU
rp375102

Background: The HPS3 Gene and Protein

The HPS3 gene encodes Hermansky-Pudlak Syndrome 3 Protein, one of several proteins whose loss-of-function mutations underlie Hermansky-Pudlak Syndrome (HPS), a rare autosomal recessive disorder. HPS is characterized by a triad of oculocutaneous albinism, bleeding diathesis due to platelet storage pool deficiency, and in some subtypes, pulmonary fibrosis or immunodeficiency.

HPS3 protein is a subunit of the Biogenesis of Lysosome-related Organelles Complex-2 (BLOC-2), together with HPS5 and HPS6. BLOC-2 plays an essential role in the proper biogenesis and trafficking of lysosome-related organelles (LROs) such as melanosomes in melanocytes and dense granules in platelets. Disruption of HPS3 function impairs the sorting and delivery of cargo proteins to these specialized organelles, resulting in the clinical manifestations of HPS-3.

At the cellular level, HPS3 participates in endosomal membrane tubulation and vesicle budding events, facilitating the transfer of membrane proteins from early endosomes to maturing LROs. Understanding the biochemical interactions of HPS3 is therefore central to elucidating the molecular basis of organelle identity and cargo sorting in eukaryotic cells.

Biochemical Properties of Recombinant Human HPS3 Protein

Recombinant Human HPS3 Protein (SKU: rp375102) is produced using recombinant expression systems to yield a high-purity protein suitable for biochemical and cell biological research applications. Recombinant production enables researchers to obtain sufficient quantities of HPS3 protein for in vitro studies that would be impractical using endogenous sources alone.

Key considerations when working with recombinant HPS3 include proper storage conditions to maintain protein stability and activity. As with other recombinant proteins, HPS3 should be handled according to the product datasheet instructions, with attention to recommended buffer composition, aliquoting strategy to minimize freeze-thaw cycles, and storage temperature.

Note: Specific physicochemical parameters such as molecular weight, purity grade, expression host, tag information, and formulation details should be confirmed against the official Aladdin Scientific product datasheet for SKU rp375102, as these specifications are subject to lot-specific variation.

Research Applications

Recombinant Human HPS3 Protein is a versatile reagent supporting a range of research applications in cell biology, biochemistry, and disease modeling:

  • Protein–Protein Interaction Studies: HPS3 can be used as bait or prey in pull-down assays and co-immunoprecipitation experiments to map its interactions within the BLOC-2 complex and with other trafficking machinery components such as Rab GTPases and SNARE proteins.
  • Structural Biology: Recombinant HPS3 provides material for X-ray crystallography, cryo-electron microscopy, or small-angle X-ray scattering (SAXS) studies aimed at resolving the three-dimensional architecture of BLOC-2.
  • Antibody Validation: Pure recombinant HPS3 serves as a positive control antigen for validating anti-HPS3 antibodies in Western blotting, ELISA, and immunofluorescence assays.
  • Biochemical Reconstitution: Researchers can incorporate recombinant HPS3 into cell-free reconstitution systems to dissect the mechanistic steps of endosomal sorting and LRO biogenesis.
  • Drug Discovery Screening: HPS3 protein can serve as a target in assays designed to identify small molecules that modulate BLOC-2 function, with potential therapeutic relevance for HPS and related lysosomal disorders.

Handling, Storage & Quality Considerations

To obtain optimal and reproducible results with Recombinant Human HPS3 Protein, adhere to the following best practices:

  • Storage: Store the protein at the temperature specified on the product datasheet (typically −20 °C or −80 °C for long-term storage). Avoid repeated freeze-thaw cycles by preparing single-use aliquots upon first thaw.
  • Handling: Thaw the protein on ice and mix gently; avoid vortexing, which can cause denaturation and aggregation. Keep protein solutions on ice during experimental setup.
  • Buffer Compatibility: Confirm that the supplied formulation buffer is compatible with downstream assay conditions. If buffer exchange is required, use a compatible desalting column or dialysis method.
  • Quality Control: Aladdin Scientific performs quality control testing on recombinant proteins. Verify the certificate of analysis (CoA) accompanying each lot for purity, identity, and biological activity data where applicable.
  • Biosafety: Handle all recombinant proteins according to institutional biosafety guidelines. Although HPS3 is a human-derived non-pathogenic protein, standard laboratory safety practices apply.

Ordering Information

Recombinant Human HPS3 Protein is available from Aladdin Scientific. Please refer to the table below for ordering details:

SKUProduct NameSupplier Reference
rp375102Recombinant Human HPS3 Proteinabsea#PP-8836

For bulk quantities, custom formulations, or additional technical support, please contact Aladdin Scientific customer service. Pricing and availability are subject to change; please consult the current Aladdin Scientific catalogue or website for the most up-to-date information.

Frequently asked questions

What is the biological function of HPS3 protein?

HPS3 is a subunit of the BLOC-2 complex (Biogenesis of Lysosome-related Organelles Complex-2), which is essential for the proper biogenesis and intracellular trafficking of lysosome-related organelles such as melanosomes and platelet dense granules. Loss-of-function mutations in HPS3 lead to Hermansky-Pudlak Syndrome type 3, characterized by albinism and bleeding tendency.

What research applications is Recombinant Human HPS3 Protein suitable for?

Recombinant Human HPS3 Protein is suitable for a variety of applications including protein–protein interaction studies (pull-down and co-immunoprecipitation), antibody validation (Western blot, ELISA, immunofluorescence), structural biology, biochemical reconstitution of endosomal sorting pathways, and small-molecule screening assays targeting BLOC-2 function.

How should Recombinant Human HPS3 Protein be stored to maintain stability?

The protein should be stored at the temperature recommended in the product datasheet, typically −20 °C or −80 °C for long-term storage. To prevent loss of activity from repeated freeze-thaw cycles, it is strongly recommended to aliquot the protein into single-use volumes immediately after the first thaw and to avoid vortexing. Always consult the certificate of analysis for lot-specific storage instructions.

What is the relationship between HPS3 and other HPS proteins?

HPS3 functions together with HPS5 and HPS6 as part of the BLOC-2 complex. There are at least ten known HPS subtypes (HPS1–HPS10), each caused by mutations in different genes encoding subunits of distinct complexes (BLOC-1, BLOC-2, BLOC-3, and AP-3). All subtypes share the core clinical features of albinism and platelet dysfunction to varying degrees, but differ in additional organ involvement.

Can Recombinant Human HPS3 Protein be used for in vivo studies?

Recombinant Human HPS3 Protein (rp375102) is produced and validated primarily for in vitro research applications. Use in in vivo or clinical settings is not supported by the current product format. Researchers intending to use this protein in cell-based or animal models should perform appropriate feasibility and safety assessments and consult the product datasheet for guidance.

References

  1. Di Pietro SM, Falcon-Perez JM, Tenza D, et al. BLOC-1 interacts with BLOC-2 and the AP-3 complex to facilitate protein trafficking on endosomes. Mol Biol Cell. 2006;17(9):4027-4038.
  2. Huizing M, Helip-Wooley A, Westbroek W, Gunay-Aygun M, Gahl WA. Disorders of lysosome-related organelle biogenesis: clinical and molecular genetics. Annu Rev Genomics Hum Genet. 2008;9:359-386.
  3. Bhatt DL, et al. (General reference: Hermansky-Pudlak Syndrome Network and OMIM entry #614072 for HPS3). Online Mendelian Inheritance in Man. OMIM #614072.

Products are supplied for research and development use only. Not for use in humans, animals, diagnosis, or therapy.

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Da — 若无特别说明,分子量单位默认为道尔顿。   Mw — 重均分子量。   Mn — 数均分子量。

产品仅供科研与开发使用,不用于人体、动物、诊断或治疗。

引用本文

阿拉丁科学.《Recombinant Human HPS3 Protein: Function & Research Use》. 阿拉丁知识库,更新于 2026年11月2日。 https://www.aladdin-e.com/zh_cn/faqs/recombinant-human-hps3-protein-en.html
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