LBR sirna

LBR siRNA (Human)

Cat. Num. AAA8237328

• Gene Names: LBR; PHA; LMN2R; TDRD18; DHCR14B
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: LBR; LBR siRNA (Human); Lamin-B receptor; Integral nuclear envelope inner membrane protein; LMN2R; LBR sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: LBR 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: 615

• Applicable Applications for LBR 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 LBR 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 LBR sirna

siRNA to inhibit LBR expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 37595750
• NCBI GeneID: 3930
• NCBI Accession #: NP_002287.2
• NCBI GenBank Nucleotide #: NM_002296.3
• UniProt Accession #: Q14739; Q14740; Q53GU7; Q59FE6; B2R5P3
• Molecular Weight: 70,703 Da
• NCBI Official Full Name: lamin-B receptor
• NCBI Official Synonym Full Names: lamin B receptor
• NCBI Official Symbol: LBR
• NCBI Official Synonym Symbols: PHA; LMN2R; TDRD18; DHCR14B
• NCBI Protein Information: lamin-B receptor
• UniProt Protein Name: Lamin-B receptor
• Protein Family: Lamin-B receptor
• UniProt Gene Name: LBR
• UniProt Entry Name: LBR_HUMAN

NCBI Description

The protein encoded by this gene belongs to the ERG4/ERG24 family. It localized in the nuclear envelope inner membrane and anchors the lamina and the heterochromatin to the membrane. It may mediate interaction between chromatin and lamin B. Mutations of this gene has been associated with autosomal recessive HEM/Greenberg skeletal dysplasia. Alternative splicing occurs at this locus and two transcript variants encoding the same protein have been identified. [provided by RefSeq, Jul 2008]

Uniprot Description

LBR: Anchors the lamina and the heterochromatin to the inner nuclear membrane. Defects in LBR are a cause of Pelger-Huet anomaly (PHA). PHA is an autosomal dominant inherited abnormality of neutrophils, characterized by reduced nuclear segmentation and an apparently looser chromatin structure. Heterozygotes show hypolobulated neutrophil nuclei with coarse chromatin. Presumed homozygous individuals have ovoid neutrophil nuclei, as well as varying degrees of developmental delay, epilepsy, and skeletal abnormalities. Defects in LBR are the cause of hydrops-ectopic calcification-moth-eaten skeletal dysplasia (HEM); also known as Greenberg skeletal dysplasia. HEM is a rare autosomal recessive chondrodystrophy characterized by early in utero lethality and, therefore, considered to be nonviable. Affected fetuses typically present with fetal hydrops, short- limbed dwarfism, and a marked disorganization of chondro-osseous calcification and may present with polydactyly and additional nonskeletal malformations. Defects in LBR may be a cause of Reynolds syndrome (REYNS). It is a syndrome specifically associating limited cutaneous systemic sclerosis and primary biliray cirrhosis. It is characterized by liver disease, telangiectasia, abrupt onset of digital paleness or cyanosis in response to cold exposure or stress (Raynaud phenomenon), and variable features of scleroderma. The liver disease is characterized by pruritis, jaundice, hepatomegaly, increased serum alkaline phosphatase and positive serum mitochondrial autoantibodies, all consistent with primary biliary cirrhosis. Belongs to the ERG4/ERG24 family.

Protein type: DNA-binding; Membrane protein, integral; Membrane protein, multi-pass

Chromosomal Location of Human Ortholog: 1q42.1

Cellular Component: nuclear membrane; membrane; integral to membrane; nuclear envelope; integral to nuclear inner membrane

Molecular Function: protein binding; DNA binding; oxidoreductase activity, acting on the CH-CH group of donors, NAD or NADP as acceptor; lamin binding

Biological Process: cholesterol biosynthetic process

Disease: Pelger-huet Anomaly; Greenberg Dysplasia; Reynolds Syndrome