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Home / Primary Antibodies / Recombinant Antibodies

LMNA Recombinant Monoclonal Antibody (CSB-RA013003A0HU)

SKU : BHA10564582

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CUSABIO TECHONOLOGY LLC

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Mfr.No.	CSB-RA013003A0HU
Alternative Names	Prelamin-A/C, Lamin-A/C, 70 kDa lamin, Renal carcinoma antigen NY-REN-32, LMNA, LMN1
Categories	Primary Antibodies
Clonality	monoclonal
Immunogen	A synthesized peptide derived from human LMNA
Involvement In Disease	Emery-Dreifuss muscular dystrophy 2, autosomal dominant (EDMD2); Emery-Dreifuss muscular dystrophy 3, autosomal recessive (EDMD3); Cardiomyopathy, dilated 1A (CMD1A); Lipodystrophy, familial partial, 2 (FPLD2); Limb-girdle muscular dystrophy 1B (LGMD1B); Charcot-Marie-Tooth disease 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 (MDCL)
Isotype	IgG
Reactivity	Human
Relevance	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. Plays an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics. Required for normal development of peripheral nervous system and skeletal muscle and for muscle satellite cell proliferation (PubMed:10080180, PubMed:22431096, PubMed:10814726, PubMed:11799477, PubMed:18551513). Required for osteoblastogenesis and bone formation (PubMed:12075506, PubMed:15317753, PubMed:18611980). Also prevents fat infiltration of muscle and bone marrow, helping to maintain the volume and strength of skeletal muscle and bone (PubMed:10587585). Required for cardiac homeostasis (PubMed:10580070, PubMed:12927431, PubMed:18611980, PubMed:23666920).
Subcellular Location	Nucleus, Nucleus envelope, Nucleus lamina, Nucleus, nucleoplasm

Antigen	LMNA
Uniprot	P02545
Gene Name	LMNA
Database Links	HGNC:6636 OMIM:115200 KEGG:hsa:4000 STRING:9606.ENSP00000357283 UniGene:Hs.594444
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. Plays an important role in nuclear assembly, chromatin organization, nuclear membrane and telomere dynamics. Required for normal development of peripheral nervous system and skeletal muscle and for muscle satellite cell proliferation
Pathway	Apoptosis
Protein Families	Intermediate filament family
Research Area	Cell Biology
Tissue Specificity	In the arteries, prelamin-A/C accumulation is not observed in young healthy vessels but is prevalent in medial vascular smooth muscle cells (VSMCs) from aged individuals and in atherosclerotic lesions, where it often colocalizes with senescent and degener

Form	Liquid
Purification	Affinity-chromatography
Storage	Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
Storage Buffer	Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.

Description	IHC ; IF ; IP ; FACS ; ELISA
Tested Application	ELISA, IHC, IF, FC, IP; Recommended dilution: IHC:1:50-1:200, IF:1:20-1:200, IP:1:200-1:1000

Datasheet
ELISA Protocol
Immunohistochemistry (IHC) Protocol
Immunoprecipitation (IP) Protocol

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SKU	Supplier No.	Size	Your price	Quantity
BHA10564582	CSB-RA013003A0HU	50ul	$210		Add to Cart
BHA10564582	CSB-RA013003A0HU	100ul	$350		Add to Cart

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