PKHD1 sirna

PKHD1 siRNA (Human)

Cat. Num. AAA8204882

• Gene Names: PKHD1; FCYT; ARPKD; TIGM1
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: PKHD1; PKHD1 siRNA (Human); FCYT; TIGM1; Fibrocystin; Polycystic kidney and hepatic disease 1 protein; Polyductin; Tigmin; PKHD1 sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: PKHD1 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: 4074

• Applicable Applications for PKHD1 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 PKHD1 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 PKHD1 sirna

siRNA to inhibit PKHD1 expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 126131102
• NCBI GeneID: 5314
• NCBI Accession #: NP_619639.3
• NCBI GenBank Nucleotide #: NM_138694.3
• UniProt Accession #: P08F94; Q5VUA2; Q5VUA3; Q5VWV1; Q86Z26; Q8TCZ9
• Molecular Weight: 371,654 Da
• NCBI Official Full Name: fibrocystin isoform 1
• NCBI Official Synonym Full Names: polycystic kidney and hepatic disease 1 (autosomal recessive)
• NCBI Official Symbol: PKHD1
• NCBI Official Synonym Symbols: FCYT; ARPKD; TIGM1
• NCBI Protein Information: fibrocystin
• UniProt Protein Name: Fibrocystin
• Protein Family: Fibrocystin
• UniProt Gene Name: PKHD1
• UniProt Synonym Gene Names: FCYT; TIGM1
• UniProt Entry Name: PKHD1_HUMAN

NCBI Description

The protein encoded by this gene is predicted to have a single transmembrane (TM)-spanning domain and multiple copies of an immunoglobulin-like plexin-transcription-factor domain. Alternative splicing results in two transcript variants encoding different isoforms. Other alternatively spliced transcripts have been described, but the full length sequences have not been determined. Several of these transcripts are predicted to encode truncated products which lack the TM and may be secreted. Mutations in this gene cause autosomal recessive polycystic kidney disease, also known as polycystic kidney and hepatic disease-1. [provided by RefSeq, Jul 2008]

Uniprot Description

PKHD1: May be required for correct bipolar cell division through the regulation of centrosome duplication and mitotic spindle assembly. May be a receptor protein that acts in collecting-duct and biliary differentiation. Defects in PKHD1 are the cause of polycystic kidney disease autosomal recessive (ARPKD). ARPKD is a severe form of polycystic kidney disease affecting the kidneys and the hepatic biliary tract. The clinical spectrum is widely variable, with most cases presenting during infancy. The fetal phenotypic features classically include enlarged and echogenic kidneys, as well as oligohydramnios secondary to a poor urine output. Up to 50% of the affected neonates die shortly after birth, as a result of severe pulmonary hypoplasia and secondary respiratory insufficiency. In the subset that survives the perinatal period, morbidity and mortality are mainly related to severe systemic hypertension, renal insufficiency, and portal hypertension due to portal-tract fibrosis. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Membrane protein, integral; Cell surface

Chromosomal Location of Human Ortholog: 6p12.2

Cellular Component: anchored to external side of plasma membrane; centrosome; perinuclear region of cytoplasm; cytoplasm; apical plasma membrane; integral to membrane

Molecular Function: protein binding; receptor activity

Biological Process: cellular calcium ion homeostasis; cell-cell adhesion; negative regulation of protein kinase B signaling cascade; homeostatic process; inhibition of NF-kappaB transcription factor; positive regulation of cell proliferation; cilium biogenesis; negative regulation of cell motility; kidney development; regulation of TOR signaling pathway; negative regulation of apoptosis

Disease: Polycystic Kidney Disease, Autosomal Recessive