Native Rabbit Alpha Skeletal Muscle Actin Protein

Cat. No.: CLPP-00150196

Product Size: 1 mg Custom size

Product Overview

Description
Native rabbit Alpha Skeletal Muscle Actin protein
Purity
> 95%
Applications
Functional Studies, SDS-PAGE
Protein Length
Full length protein
Animal Free
No
Nature
Native
Species
Rabbit
Form
Lyophilized
Sequence
MCDEDETTALVCDNGSGLVKAGFAGDDAPRAVFPSIVGRPRHQGVMVGMGQKDSYVGDEAQSKRGILTLKYPIEHGIITNWDDMEKIWHHTFYNELRVAPEEHPTLLTEAPLNPKANREKMTQIMFETFNVPAMYVAIQAVLSLYASGRTTGIVLDSGDGVTHNVPIYEGYALPHAIMRLDLAGRDLTDYLMKILTERGYSFVTTAEREIVRDIKEKLCYVALDFENEMATAASSSSLEKSYELPDGQVITIGNERFRCPETLFQPSFIGMESAGIHETTYNSIMKCDIDIRKDLYANNVMSGGTTMYPGIADRMQKEITALAPSTMKIKIIAPPERKYSVWIGGSILASLSTFQQMWITKQEYDEAGPSIVHRKCF
Sequence Similarities
Belongs to the actin family.
Predicted Molecular Weight
43 kDa

Target Information

Protein Name
ACTA1
UniProt No.
Alternative Names
a actin; ACTA; ACTA1; Actin, alpha skeletal muscle; ACTS_HUMAN; Alpha actin 1; Alpha-actin-1; ASMA; MPFD; Skeletal muscle alpha actin 1
Protein Function
Actins are highly conserved proteins that are involved in various types of cell motility and are ubiquitously expressed in all eukaryotic cells.
Involvement in Disease
Defects in ACTA1 are the cause of nemaline myopathy type 3 (NEM3). A form of nemaline myopathy. Nemaline myopathies are muscular disorders characterized by muscle weakness of varying severity and onset, and abnormal thread-or rod-like structures in muscle fibers on histologic examination. The phenotype at histological level is variable. Some patients present areas devoid of oxidative activity containg (cores) within myofibers. Core lesions are unstructured and poorly circumscribed.Defects in ACTA1 are a cause of myopathy congenital with excess of thin myofilaments (MPCETM). A congenital muscular disorder characterized at histological level by areas of sarcoplasm devoid of normal myofibrils and mitochondria, and replaced with dense masses of thin filaments. Central cores, rods, ragged red fibers, and necrosis are absent.Defects in ACTA1 are a cause of congenital myopathy with fiber-type disproportion (CFTD); also known as congenital fiber-type disproportion myopathy (CFTDM). CFTD is a genetically heterogeneous disorder in which there is relative hypotrophy of type 1 muscle fibers compared to type 2 fibers on skeletal muscle biopsy. However, these findings are not specific and can be found in many different myopathic and neuropathic conditions.

Shipping & Handling

Shipping
Shipped at 4 °C.
Storage
Store at -20 °C.

For Research Use Only. Not For Clinical Use.

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