HPS4 sirna

HPS4 siRNA (Human)

Cat. Num. AAA8228857

• Gene Names: HPS4; LE
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: HPS4; HPS4 siRNA (Human); KIAA1667; Hermansky-Pudlak syndrome 4 protein; Light-ear protein homolog; HPS4 sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: HPS4 siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
• Purity/Purification: > 97%
• Form/Format: Lyophilized powder
• Sequence Length: 708

• Applicable Applications for HPS4 sirna: RNA Interference (RNAi)
• Quality Control: Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
• Directions for Use: We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
• Components: We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human HPS4 gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
• Preparation and Storage: Shipped at 4 degree C. Store at -20 degree C for one year.

Related Product Information for HPS4 sirna

siRNA to inhibit HPS4 expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 23110966
• NCBI GeneID: 89781
• NCBI Accession #: NP_071364.4
• NCBI GenBank Nucleotide #: NM_022081.5
• UniProt Accession #: Q9NQG7; Q5H8V6; Q96LX6; Q9BY93; Q9UH37; Q9UH38; B1AHQ4
• Molecular Weight: 78,648 Da
• NCBI Official Full Name: Hermansky-Pudlak syndrome 4 protein isoform a
• NCBI Official Synonym Full Names: Hermansky-Pudlak syndrome 4
• NCBI Official Symbol: HPS4
• NCBI Official Synonym Symbols: LE
• NCBI Protein Information: Hermansky-Pudlak syndrome 4 protein
• UniProt Protein Name: Hermansky-Pudlak syndrome 4 protein
• Protein Family: Hermansky-Pudlak syndrome 4 protein
• UniProt Gene Name: HPS4
• UniProt Synonym Gene Names: KIAA1667
• UniProt Entry Name: HPS4_HUMAN

NCBI Description

This gene encodes a protein component of biogenesis of lysosome-related organelles complexes (BLOC). BLOC complexes are important for the formation of endosomal-lysosomal organelles such as melanosomes and platelet dense granules. Mutations in this gene result in subtype 4 of Hermansky-Pudlak syndrome, a form of albinism. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Aug 2012]

Uniprot Description

HPS4: May function in the pathway of organelle biogenesis. Defects in HPS4 are the cause of Hermansky-Pudlak syndrome type 4 (HPS4). 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. 4 isoforms of the human protein are produced by alternative splicing.

Chromosomal Location of Human Ortholog: 22cen-q12.3

Cellular Component: platelet dense granule; membrane; lysosome; cytoplasm; melanosome

Molecular Function: protein dimerization activity; protein binding; protein homodimerization activity

Biological Process: protein stabilization; positive regulation of eye pigmentation; lysosome organization and biogenesis; hemostasis; melanocyte differentiation; blood coagulation; protein targeting

Disease: Hermansky-pudlak Syndrome 4