Product Overview
Description
CLPP-00150817 is recombinant human LMNA protein
Applications
SDS-PAGE, Western Blot
Protein Length
Full length protein
Nature
Recombinant Protein
Sequence
METPSQRRATRSGAQASSTPLSPTRITRLQEKEDLQELNDRLAVYIDRVRSLETENAGLRLRITESEEVVSREVSGIKAAYEAELGDARKTLDSVAKERARLQLELSKVREEFKELKARNTKKEGDLIAAQARLKDLEALLNSKEAALSTALSEKRTLEGELHDLRGQVAKLEAALGEAKKQLQDEMLRRVDAENRLQTMKEELDFQKNIYSEELRETKRRHETRLVEIDNGKQREFESRLADALQELRAQHEDQVEQYKKELEKTYSAKLDNARQSAERNSNLVGAAHEELQQSRIRIDSLSAQLSQLQKQLAAKEAKLRDLEDSLARERDTSRRLLAEKEREMAEMRARMQQQLDEYQELLDIKLALDMEIHAYRKLLEGEEERLRLSPSPTSQRSRGRASSHSSQTQGGGSVTKKRKLESTESRSSFSQHARTSGRVAVEEVDEEGKFVRLRNKSNEDQSMGNWQIKRQNGDDPLLTYRFPPKFTLKAGQVVTIWAAGAGATHSPPTDLVWKAQNTWGCGNSLRTALINSTGEEVAMRKLVRSVTVVEDDEDEDGDDLLHHHHGSHCSSSGDPAEYNLRSRTVLCGTCGQPADKASASGSGAQVGGPISSGSSASSVTVTRSYRSVGGSGGGSFGDNLVTRS
Sequence Similarities
Belongs to the intermediate filament family.
Target Information
Alternative Names
70 kDa lamin; CDDC; EMD2; FPL; FPLD; HGPS; IDC; LAMIN A; lamin A/C; LAMIN C; Lamin-A/C; LDP1; LFP; LMN 1; LMN A; LMN C; LMNA; LMNA_HUMAN; LMNC; PRO1; Renal carcinoma antigen NY-REN-32
Protein Function
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. Play an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics.Prelamin-A/C can accelerate smooth muscle cell senescence. It acts to disrupt mitosis and induce DNA damage in vascular smooth muscle cells (VSMCs), leading to mitotic failure, genomic instability, and premature senescence.
Tissue Specificity
In the arteries, prelamin-A/C accumulation is not observed in young healthy vessels but is prevalent in medial vascular smooth muscle celle (VSMCs) from aged individuals and in atherosclerotic lesions, where it often colocalizes with senescent and degenerate VSMCs. Prelamin-A/C expression increases with age and disease. In normal aging, the accumulation of prelamin-A/C is caused in part by the down-regulation of ZMPSTE24/FACE1 in response to oxidative stress.
Involvement in Disease
Defects in LMNA are the cause of Emery-Dreifuss muscular dystrophy type 2 (EDMD2); cardiomyopathy dilated type 1A (CMD1A); familial partial lipodystrophy type 2 (FPLD2); limb-girdle muscular dystrophy type 1B (LGMD1B); Charcot-Marie-Tooth disease type 2B1 (CMT2B1); Hutchinson-Gilford progeria syndrome (HGPS); cardiomyopathy dilated with hypergonadotropic hypogonadism (CMDHH); mandibuloacral dysplasia with type A lipodystrophy (MADA); lethal tight skin contracture syndrome (LTSCS); heart-hand syndrome Slovenian type (HHS-Slovenian); muscular dystrophy congenital LMNA-related (CMD-LMNA).
Shipping & Handling
Constituents
20% Glycerol, 0.003% Sodium chloride, Phosphate Buffer, 0.015% DTT, 0.044% EDTA.
Shipping
Shipped at 4 °C.
