lamin B receptor, ELISA Kit

Mouse Lamin-B receptor (LBR) ELISA kit; DHCR14B; FLJ43126; LMN2R; MGC9041; PHA; integral nuclear envelope inner membrane protein; lamin B receptor

For Research Use Only. Not for use in diagnostic procedures.

Store all reagents at 2-8 degree C

Select online data sheet information is drawn from bioinformatics databases, occasionally resulting in ambiguous or non-relevant product information. It is the responsibility of the customer to review, verify, and evaluate the information to make sure it matches their requirements before purchasing the kit. Our ELISA Kit assays are dynamic research tools and sometimes they may be updated and improved. If the format of this assay is important to you then please request the current manual or contact our technical support team with a presales inquiry before placing an order. We will confirm the current details of the assay. We cannot guarantee the sample manual posted online is the most current manual.

Small volumes of LBR elisa kit vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

MBS9341817 is a ready-to-use microwell, strip plate ELISA (enzyme-linked immunosorbent assay) Kit for analyzing the presence of the lamin B receptor (LBR) ELISA Kit target analytes in biological samples. The concentration gradients of the kit standards or positive controls render a theoretical kit detection range in biological research samples containing LBR. The ELISA analytical biochemical technique of the MBS9341817 kit is based on LBR antibody-LBR antigen interactions (immunosorbency) and an HRP colorimetric detection system to detect LBR antigen targets in samples. The ELISA Kit is designed to detect native, not recombinant, LBR. Appropriate sample types may include undiluted body fluids and/or tissue homogenates, secretions. Quality control assays assessing reproducibility identified the intra-assay CV (%) and inter-assay CV(%).

71,440 Da

lamin B receptor

lamin-B receptor; ichthyosis; integral nuclear envelope inner membrane protein

Lamin-B receptor

LBR_MOUSE

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

Cellular Component: nuclear lamina; nuclear membrane; membrane; cytoplasm; integral to membrane; nuclear inner membrane; nuclear pore; nuclear envelope; nucleus

Molecular Function: DNA binding; oxidoreductase activity, acting on the CH-CH group of donors, NAD or NADP as acceptor; chaperone binding; nuclear localization sequence binding

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