Human Hermansky Pudlak Syndrome Protein 6 (HPS6) ELISA Kit | HPS6 elisa kit

Human Hermansky Pudlak Syndrome Protein 6 (HPS6) ELISA Kit

Cat. Num. AAA2023414

• Reactivity: Human
• Synonyms: Hermansky Pudlak Syndrome Protein 6 (HPS6); Human Hermansky Pudlak Syndrome Protein 6 (HPS6) ELISA Kit; Ru; Ruby-eye protein homolog; HPS6 elisa kit

• Reactivity: Human
• Specificity: This assay has high sensitivity and excellent specificity for detection of Hermansky Pudlak Syndrome Protein 6 (HPS6).; No significant cross-reactivity or interference between Hermansky Pudlak Syndrome Protein 6 (HPS6) and analogues was observed.
• Sequence Length: 775

• Assay Type: Double-antibody Sandwich
• Samples: Tissue homogenates and Other Biological Fluids
• Detection Range: 0.156-10ng/mL
• Sensitivity: < 0.054ng/mL
• Application: Enzyme-linked immunosorbent assay for Antigen Detection.
• Intra-assay Precision (Precision within an assay): 3 samples with low, middle and high level Hermansky Pudlak Syndrome Protein 6 (HPS6) were tested 20 times on one plate, respectively.
• Inter-assay Precision (Precision between assays): 3 samples with low, middle and high level Hermansky Pudlak Syndrome Protein 6 (HPS6) were tested on 3 different plates, 8 replicates in each plate.
• CV(%) =: SD/meanX100
• Intra-Assay: CV<10%
• Inter-Assay: CV<12%
• Preparation and Storage: The stability of kit is determined by the loss rate of activity. The loss rate of this kit is less than 5% within the expiration date under appropriate storage condition. ; To minimize extra influence on the performance, operation procedures and lab conditions, especially room temperature, air humidity, incubator temperature should be strictly controlled. It is also strongly suggested that the whole assay is performed by the same operator from the beginning to the end.

Typical Testing Data/Standard Curve (for reference only)

Related Product Information for HPS6 elisa kit

The test principle applied in this kit is Sandwich enzyme immunoassay. The microtiter plate provided in this kit has been pre-coated with an antibody specific to Hermansky Pudlak Syndrome Protein 6 (HPS6). Standards or samples are then added to the appropriate microtiter plate wells with a biotin-conjugated antibody specific to Hermansky Pudlak Syndrome Protein 6 (HPS6). Next, Avidin conjugated to Horseradish Peroxidase (HRP) is added to each microplate well and incubated. After TMB substrate solution is added, only those wells that contain Hermansky Pudlak Syndrome Protein 6 (HPS6), biotin-conjugated antibody and enzyme-conjugated Avidin will exhibit a change in color. The enzyme-substrate reaction is terminated by the addition of sulphuric acid solution and the color change is measured spectrophotometrically at a wavelength of 450nm ± 10nm. The concentration of Hermansky Pudlak Syndrome Protein 6 (HPS6) in the samples is then determined by comparing the O.D. of the samples to the standard curve.

Product Categories/Family for HPS6 elisa kit

Neuroscience

NCBI and Uniprot Product Information

• NCBI GI #: 31881785
• NCBI GeneID: 79803
• NCBI Accession #: NP_079023.2
• NCBI GenBank Nucleotide #: NM_024747.5
• UniProt Accession #: Q86YV9; Q5VV69; Q9H685
• Molecular Weight: 82,975 Da
• NCBI Official Full Name: Hermansky-Pudlak syndrome 6 protein
• NCBI Official Synonym Full Names: Hermansky-Pudlak syndrome 6
• NCBI Official Symbol: HPS6
• NCBI Protein Information: Hermansky-Pudlak syndrome 6 protein
• UniProt Protein Name: Hermansky-Pudlak syndrome 6 protein
• Protein Family: Hermansky-Pudlak syndrome 6 protein
• UniProt Gene Name: HPS6
• UniProt Synonym Gene Names: Ru
• UniProt Entry Name: HPS6_HUMAN

NCBI Description

This intronless 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 5 protein. Mutations in this gene are associated with Hermansky-Pudlak syndrome type 6. [provided by RefSeq, Jul 2008]

Uniprot Description

HPS6: May regulate the synthesis and function of lysosomes and of highly specialized organelles, such as melanosomes and platelet dense granules. Defects in HPS6 are the cause of Hermansky-Pudlak syndrome type 6 (HPS6). 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.

Protein type: Unknown function

Chromosomal Location of Human Ortholog: 10q24.32

Cellular Component: membrane; endoplasmic reticulum; early endosome membrane

Molecular Function: GTP-dependent protein binding; Rab GTPase binding

Biological Process: organelle organization and biogenesis; melanocyte differentiation; blood coagulation

Disease: Hermansky-pudlak Syndrome 6