Western Blot (WB)
(Western blot analysis of extracts of various cell lines, using LBR antibody.)
Rabbit anti-Human LBR Polyclonal Antibody | anti-LBR antibody
LBR Antibody
Immunohistochemistry: 1:50 - 1:200
Western Blot (WB)
(Western blot analysis of extracts of various cell lines, using LBR antibody.)
Western Blot (WB)
(Western blot analysis of extracts of various cell lines, using LBR antibody.)
NCBI and Uniprot Product Information
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
Research Articles on LBR
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Product Notes
The LBR lbr (Catalog #AAA9412806) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The LBR Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's LBR can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB), Immunohistochemistry (IHC). Western blotting: 1:500 - 1:2000 Immunohistochemistry: 1:50 - 1:200. Researchers should empirically determine the suitability of the LBR lbr for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "LBR, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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