Product Name	Anti-HPS6/AF488
Chinese Name	AF488标记的Hermansky-Pudlak综合征蛋白6抗体
Alias	FLJ22501; Hermansky Pudlak syndrome 6 protein; Hermansky-Pudlak syndrome 6 protein; HPS6; HPS6_HUMAN; MGC20522; Ru; Ruby eye protein homolog; Ruby-eye protein homolog.
Research Area	immunology  Signal transduction  Binding protein
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Human,  (predicted: Mouse, Rat, Pig, Cow, Horse, )
Applications	ICC=1:50-200 IF=1:50-200  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Molecular weight	83kDa
Form	Lyophilized or Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human HPS6
Lsotype	IgG
Purification	affinity purified by Protein A
Storage Buffer	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
Storage	Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
Product Detail	background: 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] Function: May regulate the synthesis and function of lysosomes and of highly specialized organelles, such as melanosomes and platelet dense granules. Subcellular Location: Microsome membrane. Cytoplasm > cytosol. Early endosome membrane. Tissue Specificity: Ubiquitous. DISEASE: Defects in HPS6 are the cause of Hermansky-Pudlak syndrome type 6 (HPS6) [MIM:614075]. 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. Database links: Entrez Gene: 79803 Human Omim: 607522 Human SwissProt: Q86YV9 Human Unigene: 125133 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

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