Recombinant Human Progerin protein (ab93918)
Key features and details
- Expression system: Escherichia coli
- Purity: > 90% SDS-PAGE
- Tags: His tag N-Terminus, DDDDK tag N-Terminus
- Suitable for: WB, SDS-PAGE
Description
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Product name
Recombinant Human Progerin protein
See all Progerin proteins and peptides -
Purity
> 90 % SDS-PAGE. -
Expression system
Escherichia coli -
Accession
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Protein length
Full length protein -
Animal free
No -
Nature
Recombinant -
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Species
Human -
Sequence
MAHHHHHHVGTGSNDDDDKSPDMETPSQRRATRSGAQASSTPLSPTRITR LQEKEDLQELNDRLAVYIDRVRSLETENAGLRLRITESEEVVSREVSGIK AAYEAELGDARKTLDSVAKERARLQLELSKVREEFKELKARNTKKEGDLI AAQARLKDLEALLNSKEAALSTALSEKRTLEGELHDLRGQVAKLEAALGE AKKQLQDEMLRRVDAENRLQTMKEELDFQKNIYSEELRETKRRHETRLVE IDNGKQREFESRLADALQELRAQHEDQVEQYKKELEKTYSAKLDNARQSA ERNSNLVGAAHEELQQSRIRIDSLSAQLSQLQKQLAAKEAKLRDLEDSLA RERDTSRRLLAEKEREMAEMRARMQQQLDEYQELLDIKLALDMEIHAYRK LLEGEEERLRLSPSPTSQRSRGRASSHSSQTQGGGSVTKKRKLESTESRS SFSQHARTSGRVAVEEVDEEGKFVRLRNKSNEDQSMGNWQIKRQNGDDPL LTYRFPPKFTLKAGQVVTIWAAGAGATHSPPTDLVWKAQNTWGCGNSLRT ALINSTGEEVAMRKLVRSVTVVEDDEDEDGDDLLHHHHGSHCSSSGDPAE YNLRSRTVLCGTCGQPADKASASGSGAQSPQNCSIM -
Amino acids
1 to 614 -
Tags
His tag N-Terminus , DDDDK tag N-Terminus
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Associated products
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Related Products
Specifications
Our Abpromise guarantee covers the use of ab93918 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
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Applications
Western blot
SDS-PAGE
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Form
Liquid -
Concentration information loading...
Preparation and Storage
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Stability and Storage
Shipped at 4°C. Upon delivery aliquot. Store at -80°C. Avoid freeze / thaw cycle.
Constituents: 20% Glycerol, 0.315% Tris HCl, 2.922% Sodium chloride, 0.015% DTT, 0.044% EDTA
General Info
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Alternative names
- 70 kDa lamin
- CDCD1
- CDDC
see all -
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. Required for osteoblastogenesis and bone formation. Also prevents fat infiltration of muscle and bone marrow, helping to maintain the volume and strength of skeletal muscle and bone.
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 cells (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
Emery-Dreifuss muscular dystrophy 2, autosomal dominant
Emery-Dreifuss muscular dystrophy 3, autosomal recessive
Cardiomyopathy, dilated 1A
Lipodystrophy, familial partial, 2
Limb-girdle muscular dystrophy 1B
Charcot-Marie-Tooth disease 2B1
Hutchinson-Gilford progeria syndrome
Cardiomyopathy, dilated, with hypergonadotropic hypogonadism
Mandibuloacral dysplasia with type A lipodystrophy
Lethal tight skin contracture syndrome
Heart-hand syndrome Slovenian type
Muscular dystrophy congenital LMNA-related
Defects in LMNA may cause a late-onset cardiocutaneous progeria syndrome characterized by cutaneous manifestations of aging appearing in the third decade of life, cardiac valve calcification and dysfunction, prominent atherosclerosis, and cardiomyopathy, leading to death on average in the fourth decade. -
Sequence similarities
Belongs to the intermediate filament family.
Contains 1 LTD domain. -
Post-translational
modificationsIncreased phosphorylation of the lamins occurs before envelope disintegration and probably plays a role in regulating lamin associations.
Proteolytic cleavage of the C-terminal of 18 residues of prelamin-A/C results in the production of lamin-A/C. The prelamin-A/C maturation pathway includes farnesylation of CAAX motif, ZMPSTE24/FACE1 mediated cleavage of the last three amino acids, methylation of the C-terminal cysteine and endoproteolytic removal of the last 15 C-terminal amino acids. Proteolytic cleavage requires prior farnesylation and methylation, and absence of these blocks cleavage.
Sumoylation is necessary for the localization to the nuclear envelope.
Farnesylation of prelamin-A/C facilitates nuclear envelope targeting. -
Cellular localization
Nucleus speckle and Nucleus. Nucleus envelope. Nucleus lamina. Nucleus, nucleoplasm. Farnesylation of prelamin-A/C facilitates nuclear envelope targeting and subsequent cleaveage by ZMPSTE24/FACE1 to remove the farnesyl group produces mature lamin-A/C, which can then be inserted into the nuclear lamina. EMD is required for proper localization of non-farnesylated prelamin-A/C. - Information by UniProt
Images
Protocols
To our knowledge, customised protocols are not required for this product. Please try the standard protocols listed below and let us know how you get on.
Datasheets and documents
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SDS download
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Datasheet download
References (0)
ab93918 has not yet been referenced specifically in any publications.