Human TPM2 Recombinant Protein (RPPB4990)

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SKU:
RPPB4990
Product type:
Recombinant Protein
Size:
5ug
Species:
Human
Target:
TPM2
Synonyms:
Tropomyosin 2 (beta)
Synonyms:
DA1
Synonyms:
TMSB
Synonyms:
DA2B
Source:
Escherichia Coli
Uniprot:
P07951
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Description

Product Name:Human TPM2 Recombinant Protein
Product Code:RPPB4990
Size:5µg
Species:Human
Target:TPM2
Synonyms:Tropomyosin 2 (beta), DA1, TMSB, DA2B, AMCD1, NEM4, Arthrogryposis Multiplex Congenital Distal-Type 1, beta-Tropomyosin.
Source:Escherichia Coli
Physical Appearance:Sterile Filtered clear solution.
Formulation:The TPM2 protein solution (0.5mg/ml) is formulated in 20mM Tris-HCl buffer (pH8.0), 1mM DTT, 100mM NaCl and 30% glycerol.
Stability:Store at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. For long term storage it is recommended to add a carrier protein (0.1% HSA or BSA).Avoid multiple freeze-thaw cycles.
Purity:Greater than 90% as determined by SDS-PAGE.
Amino Acid Sequence:MGSSHHHHHH SSGLVPRGSH MDAIKKKMQM LKLDKENAID RAEQAEADKK QAEDRCKQLE EEQQALQKKL KGTEDEVEKY SESVKEAQEK LEQAEKKATD AEADVASLNR RIQLVEEELD RAQERLATAL QKLEEAEKAA DESERGMKVI ENRAMKDEEK MELQEMQLKE AKHIAEDSDR KYEEVARKLV ILEGELERSE ERAEVAESRA RQLEEELRTM DQALKSLMAS EEEYSTKEDK YEEEIKLLEE KLKEAETRAE FAERSVAKLE KTIDDLEETL ASAKEENVEI HQTLDQTLLE LNNL

Tropomyosin beta chain isoform 2 is affiliate to the actin filament binding protein family which primarily expressed in slow, type 1 muscle fibers. Mutations in TPM2 can change the expression of other sarcomeric tropomyosin proteins, and cause cap disease, nemaline myopathy and distal arthrogryposis syndromes.

TPM2 produced in E.Coli is a single, non-glycosylated polypeptide chain containing 304 amino acids (1-284a.a.) and having a molecular mass of 35.1kDa(molecular weight on SDS-PAGE will appear higher).TPM2 is fused to a 20 amino acid His-tag at N-terminus & purified by proprietary chromatographic techniques.

UniProt Protein Function:TPM2: Binds to actin filaments in muscle and non-muscle cells. Plays a central role, in association with the troponin complex, in the calcium dependent regulation of vertebrate striated muscle contraction. Smooth muscle contraction is regulated by interaction with caldesmon. In non-muscle cells is implicated in stabilizing cytoskeleton actin filaments. The non-muscle isoform may have a role in agonist-mediated receptor internalization. Defects in TPM2 are the cause of nemaline myopathy type 4 (NEM4). 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. Nemaline myopathy type 4 presents from infancy to childhood with hypotonia and moderate-to-severe proximal weakness with minimal or no progression. Major motor milestones are delayed but independent ambulation is usually achieved, although a wheelchair may be needed in later life. Defects in TPM2 are the cause of distal arthrogryposis type 1A (DA1A). A form of distal arthrogryposis, a disease characterized by congenital joint flexures or contractures that mainly involve the distal parts of the limbs, and affect two or more different body areas in the absence of a primary neurological or muscle disease. Distal arthrogryposis type 1 is characterized largely by camptodactyly and clubfoot. Hypoplasia and/or absence of some interphalangeal creases is common. The shoulders and hips are less frequently affected. Belongs to the tropomyosin family. 3 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:

Protein type:Motility/polarity/chemotaxis; Motor

Chromosomal Location of Human Ortholog: 9p13

Cellular Component: cytosol; muscle thin filament tropomyosin

Molecular Function:actin binding; structural constituent of muscle

Biological Process: muscle contraction; muscle filament sliding; regulation of ATPase activity

Disease: Arthrogryposis, Distal, Type 1a; Arthrogryposis, Distal, Type 2b; Myopathy, Congenital, With Fiber-type Disproportion; Nemaline Myopathy 4

NCBI Summary:This gene encodes beta-tropomyosin, a member of the actin filament binding protein family, and mainly expressed in slow, type 1 muscle fibers. Mutations in this gene can alter the expression of other sarcomeric tropomyosin proteins, and cause cap disease, nemaline myopathy and distal arthrogryposis syndromes. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Mar 2009]
UniProt Code:P07951
NCBI GenInfo Identifier:136090
NCBI Gene ID:7169
NCBI Accession:P07951.1
UniProt Secondary Accession:P07951,P06468, Q13894, Q53FM4, Q5TCU4, Q5TCU7, Q9UH67 A6NM85,
UniProt Related Accession:P07951
Molecular Weight:28,684 Da
NCBI Full Name:Tropomyosin beta chain
NCBI Synonym Full Names:tropomyosin 2 (beta)
NCBI Official Symbol:TPM2  
NCBI Official Synonym Symbols:DA1; DA2B; NEM4; TMSB; AMCD1; HEL-S-273  
NCBI Protein Information:tropomyosin beta chain
UniProt Protein Name:Tropomyosin beta chain
UniProt Synonym Protein Names:Beta-tropomyosin; Tropomyosin-2
Protein Family:Tropomyosin
UniProt Gene Name:TPM2  
UniProt Entry Name:TPM2_HUMAN
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