BD-PN1454

TNNT1 Rabbit Polyclonal Antibody

常规抗体

TNNT1 TNT

Troponin T, slow skeletal muscle (TnTs) (Slow skeletal muscle troponin T) (sTnT)

WB

Human,Mouse,Rat

1 mg/ml

Liquid in PBS containing 50% glycerol, and 0.02% New type preservative N.

7138

P13805

https://www.uniprot.org/uniprotkb/P13805/entry

O88346

http://www.uniprot.org/uniprot/O88346

Q7TNB2

http://www.uniprot.org/uniprot/O54941Q7TNB2

Synthesized peptide derived from part region of human protein

TNNT1 Polyclonal Antibody detects endogenous levels of protein.

WB 1:500-2000 ELISA 1:5000-20000

30kD

-20°C/1 year

Polyclonal, Rabbit,IgG

This gene encodes a protein that is a subunit of troponin, which is a regulatory complex located on the thin filament of the sarcomere. This complex regulates striated muscle contraction in response to fluctuations in intracellular calcium concentration. This complex is composed of three subunits: troponin C, which binds calcium, troponin T, which binds tropomyosin, and troponin I, which is an inhibitory subunit. This protein is the slow skeletal troponin T subunit. Mutations in this gene cause nemaline myopathy type 5, also known as Amish nemaline myopathy, a neuromuscular disorder characterized by muscle weakness and rod-shaped, or nemaline, inclusions in skeletal muscle fibers which affects infants, resulting in death due to respiratory insufficiency, usually in the second year. Multiple transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Ju

Skeletal muscle,

cytosol,troponin complex,

disease:Defects in TNNT1 are the cause of nemaline myopathy type 5 (NEM5) [MIM:605355]; also known as Amish nemaline myopathy (ANM) [MIM:605355]. This form of nemaline myopathy (NEM) is common among Old Order Amish with an incidence of approximately 1:500. Affected infants display tremors with hypotonia and mild contractures of the shoulders and hips. Proximal contractures progressively weaken and a pectus carinatum deformity develops before children die of respiratory insufficiency, usually in the second year. A nucleotide replacement in exon 11 causes the protein to be truncated after residue 178.,function:Troponin T is the tropomyosin-binding subunit of troponin, the thin filament regulatory complex which confers calcium-sensitivity to striated muscle actomyosin ATPase activity.,similarity:Belongs to the troponin T family.,

现货

The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.