货号：MAB3540

品牌：Merck Millipore

规格：100Ug

目录价：￥6526.00

市场价格：￥5547.10

会员价格：￥5220.80

金山科研平台，产品价格货期咨询微信：jinshanbio Description: Anti-Lamin A Antibody, C-terminus, a.a. 598-611, clone 133A2 | MAB3540 View All» Promotional Text: Special Shipping Offer on Antibodies100% Performance Guaranteed View All» Trade Name: Chemicon (Millipore) View All» Specificity: Monoclonal 133A2 reacts against human lamin A but not lamin C. The epitope lies with the 98 amino acids of the C terminus that is unique to lamin A. Specifically deletion analysis has shown that amino acids 598-611 were essential for reactivity {Hozak, P et.al. (1995), J. Cell Sci 635-644}. View All» Epitope: C-terminus, a.a. 598-611 View All» Immunogen: Synthetic peptide from the C-terminus of human lamin A. This sequence is not present in the the lamin C isoform. View All» Clone: 133A2 View All» Isotype: IgG3 View All» Background Information: Nuclear lamins form a network of filaments at the nucleoplasmic site of the nuclear membrane. Two main subtypes of nuclear lamins can be distinguished: A-type lamins and B-type lamins. The A-type lamins comprise a set of three proteins arising from the same gene by alternative splicing, i.e. lamin A, lamin C and lamin Adel 10, while the B-type lamins include two proteins arising from two distinct genes, i.e. lamin B1 and lamin B2. View All» Species Reactivity:

• Bovine

• Canine

• Human

• Mouse

• Rat
  View All» Application Notes: Immunoblotting: Lamin A detected as a 70kDa protein under reduced conditions. Immunocytochemistry on fixed cells (methanol/acetone fixation) Immunohistochemistry on frozen tissue sections. Optimal working dilutions must be determined by the end user. View All» Presentation: Liquid in buffer containing 0.1% sodium azide. View All» Storage Conditions: Maintain -20°C in undiluted aliquots for up to 6 months. Avoid repeated freeze/thaw cycles. View All» UniProt Number: P02545 View All» Entrez Gene Number:
  • NM_005572.3

  • NM_170707.2

  • NM_170708.2
    View All» Gene Symbol:
    • LMNA

    • HGPS

    • EMD2

    • FPLD

    • CDCD1

    • LDP1

    • LGMD1B

    • IDC

    • CMT2B1

    • FPL

    • LMNC

    • PRO1

    • Lamin-A/C

    • LMN1

    • CMD1A

    • LFP

    • CDDC
      View All» Alternate Names: 70 kDa Lamin View All» Usage Statement: Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals. View All» Key Applications:
      • Flow Cytometry

      • Western Blotting

      • Immunocytochemistry

      • Immunohistochemistry
        View All» Entrez Gene Summary: The nuclear lamina consists of a two-dimensional matrix of proteins located next to the inner nuclear membrane. The lamin family of proteins make up the matrix and are highly conserved in evolution. During mitosis, the lamina matrix is reversibly disassembled as the lamin proteins are phosphorylated. Lamin proteins are thought to be involved in nuclear stability, chromatin structure and gene expression. Vertebrate lamins consist of two types, A and B. Through alternate splicing, this gene encodes three type A lamin isoforms. Mutations in this gene lead to several diseases: Emery-Dreifuss muscular dystrophy, familial partial lipodystrophy, limb girdle muscular dystrophy, dilated cardiomyopathy, Charcot-Marie-Tooth disease, and Hutchinson-Gilford progeria syndrome. View All» UniProt Summary: FUNCTION: SwissProt: P02545 # Lamins are components of the nuclear lamina, a fibrous layer on the nucleoplasmic side of the inner nuclear membrane, which is thought to provide a framework for the nuclear envelope and may also interact with chromatin. Lamin A and C are present in equal amounts in the lamina of mammals.SIZE: 664 amino acids; 74139 Da SUBUNIT: Homodimer of lamin A and lamin C. Interacts with lamin- associated polypeptides IA, IB and TMPO-alpha, RB1 and with emerin. Proteolytically processed isoform A interacts with NARF.SUBCELLULAR LOCATION: Nucleus.PTM: Increased phosphorylation of the lamins occurs before envelope disintegration and probably plays a role in regulating lamin associations. & The C-terminal 18 residues are removed by proteolytic cleavage in isoform A. Proteolytic cleavage requires prior farnesylation and absence of farnesylation blocks cleavage (By similarity).DISEASE: SwissProt: P02545 # Defects in LMNA are a cause of Emery-Dreifuss muscular dystrophy type 2 (EDMD2) [MIM:181350]. EDMD2 is an autosomal dominant disorder characterized by slowly progressive muscle wasting and weakness, early contractures of the elbows Achilles tendons and spine, and cardiomyopathy associated with cardiac conduction defects. & Defects in LMNA are a cause of Emery-Dreifuss muscular dystrophy type 3 (EDMD3) [MIM:604929]. EDMD3 is an autosomal recessive disorder characterized by early contractures, muscle wasting and weakness and cardiomyopathy. & Defects in LMNA are a cause of dilated cardiomyopathy 1A (CMD1A) [MIM:115200]. Dilated cardiomyopathy, a disorder characterized by cardiac dilation and reduced systolic function, represents an outcome of a heterogeneous group of inherited and acquired disorders. & Defects in LMNA are a cause of CMD1A with quadriceps myopathy [MIM:607920]. Inheritance is autosomal dominant and the phenotype severe. & Defects in LMNA are a cause of generalized lipoatrophy associated with diabetes, hepatic steatosis, hypertrophic cardiomyopathy and leukomelanodermic papules (LDHCP) [MIM:608056]. LDHCP is a disorder characterized by acquired generalized lipoatrophy with metabolic alterations, massive liver steatosis, distinctive cutaneous manifestations, and cardiac abnormalities involving both endocardium and myocardium. & Defects in LMNA are a cause of familial partial lipodystrophy (FPLD) [MIM:151660]; also known as familial partial lipodystrophy Dunnigan type. FPLD is an autosomal dominant disorder characterized by marked loss of subcutaneous adipose tissue from the extremities and trunk but by excess fat deposition in the head and neck. Frequently associated with profound insulin resistance, dyslipidemia, and diabetes. & Defects in LMNA are the cause of limb-girdle muscular dystrophy type 1B (LGMD1B) [MIM:159001]. LGMD1B is an autosomal dominantly inherited slowly progressive limb girdle muscular dystrophy, with age-related atrioventricular cardiac conduction disturbances and the absence of early contractures. & Defects in LMNA are the cause of Charcot-Marie-Tooth disease type 2B1 (CMT2B1) [MIM:605588]. CMT2B1 is a form of Charcot-Marie-Tooth disease, the most common inherited disorder of the peripheral nervous system. Charcot-Marie-Tooth disease is classified in two main groups on the basis of electrophysiologic properties and histopathology: primary peripheral demyelinating neuropathy or CMT1, and primary peripheral axonal neuropathy or CMT2. Neuropathies of the CMT2 group are characterized by signs of axonal regeneration in the absence of obvious myelin alterations, normal or slightly reduced nerve conduction velocities, and progressive distal muscle weakness and atrophy. CMT2B1 inheritance is autosomal recessive. & Defects in LMNA are the cause of Hutchinson-Gilford progeria syndrome (HGPS) [MIM:176670]. HGPS is a rare genetic disorder characterized by features reminiscent of marked premature aging. & Defects in LMNA are a cause of familial atrial fibrillation (ATFB) [MIM:607554]. Atrial fibrillation is a cardiac arrhythmia characterized by rapid and irregular activation of the atrium. It causes thromboembolism, tachycardia-mediated cardiomyopathy and heart failure. & Defects in LMNA are a cause of Werner syndrome (WRN) [MIM:277700]. WRN is an autosomal, recessively inherited, segmental progeroid syndrome, in which multiple aspects (or segments) of aging phenotypes seem to be entailed. The features of Werner syndrome are scleroderma-like skin changes, especially in the extremities, cataract, subcutaneous calcification, premature arteriosclerosis, diabetes mellitus, and a wizened and prematurely aged facies. & Defects in LMNA are the cause of mandibuloacral dysplasia with type A lipodystrophy (MADA) [MIM:248370]. Mandibuloacral dysplasia (MAD) is a rare autosomal recessive disorder characterized by mandibular and clavicular hypoplasia, acroosteolysis, delayed closure of the cranial suture, joint contractures, and types A or B patterns of lipodystrophy. Type A lipodystrophy observed in MADA, is characterized by fat loss restricted to the extremities. & Defects in LMNA are a cause of lethal tight skin contracture syndrome [MIM:275210]; also called restrictive dermopathy (RD). Lethal tight skin contracture syndrome is a rare disorder mainly characterized by intrauterine growth retardation, tight and rigid skin with erosions, prominent superficial vasculature and epidermal hyperkeratosis, facial features (small mouth, small pinched nose and micrognathia), sparse/absent eyelashes and eyebrows, mineralization defects of the skull, thin dysplastic clavicles, pulmonary hypoplasia, multiple joint contractures and an early neonatal lethal course. Liveborn children usually die within the first week of life. The overall prevalence of consanguineous cases suggested an autosomal recessive inheritance.SIMILARITY: SwissProt: P02545 ## Belongs to the intermediate filament family.MISCELLANEOUS: There are three types of lamins in human cells: A, B, and C. & The structural integrity of the lamina is strictly controlled by the cell cycle, as seen by the disintegration and formation of the nuclear envelope in prophase and telophase, respectively. View All» Brand Family: Chemicon View All» Product Name: Anti-Lamin A Antibody, C-terminus, a.a. 598-611, clone 133A2 | MAB3540 View All» Concentration: 1 mg/mL View All» Antibody Type: Monoclonal Antibody View All» Qty/Pk: 100 µg View All» Format: Purified View All» Host: Mouse View All»
        © 金山科研平台是专业的授权总代理区域代理经销平台。
        © 如需询价，请加客服QQ：1749072012 、客服微信：jinshanbio，或发送邮件到1749072012@qq.com
        © 平台为生命科学研究相关领域提供一站式耗材试剂仪器解决方案和采购服务，数据资源基于CC协议。
        © 本文地址：https://www.16ao.com/thread-131205.htm
        © Merck Millipore代理MAB3540 Anti-Lamin A Antibody, CT, a.a. 598-611, clone 133A2;store at -20℃，产品报价联系微信jinshanbio