GOAT ANTI HUMAN DESMIN (C-TERMINAL) | MyBioSource MBS223525 product information

product summary

company name :

MyBioSource

product type :

antibody

product name :

GOAT ANTI HUMAN DESMIN (C-TERMINAL)

catalog :

MBS223525

quantity :

0.1 mg

price :

500 USD

clonality :

polyclonal

host :

goat

conjugate :

nonconjugated

reactivity :

human, mouse, rat, dog, cow

application :

western blot, ELISA, enzyme immunoassay

more info or order :

MyBioSource product webpage

product information

catalog number :

MBS223525

products type :

Antibody

products full name :

GOAT ANTI HUMAN DESMIN (C-TERMINAL)

products short name :

DESMIN

other names :

desmin; Desmin; desmin; intermediate filament protein; mutant desmin p.K241E; desmin

other gene names :

DES; DES; CSM1; CSM2; LGMD2R

uniprot entry name :

DESM_HUMAN

clonality :

Polyclonal

isotype :

IgG

host :

Goat

reactivity :

Bovine (Expected from Sequence), Dog (Expected from Sequence), Mouse, Pig (Expected from Sequence), Rat

sequence length :

470

form :

Purified. Purified IgG - liquid

concentration :

IgG concentration 0.5 mg/ml

storage stability :

Store at 4 degree C or at -20 degree C if preferred. Storage in frost-free freezers is not recommended. This product should be stored undiluted. Avoid repeated freezing and thawing as this may denature the antibody. Should this product contain a precipitate we recommend microcentrifugation before use. Shelf Life: 18 months from date of despatch.

tested application :

ELISA (EIA), Immunohistology Paraffin*, Western Blot (WB)

app notes :

Western Blot: Goat anti Human Desmin detects a band of approximately 55kDa in human skeletal muscle cell lysates. ELISA: Minimum Dilution: 1/2000. Immunohistology - Paraffin: Minimum Dilution: 3.0; Maximum Dilution: 6.0ug/ml; Application Note: This product requires heat-mediated antigen retrieval prior to staining of paraffin sections. Sodium citrate buffer pH6.0 is recommended for this purpose. . Western Blotting: Minimum Dilution: 0.1; Maximum Dilution: 0.3ug/ml

other info1 :

Perservative Stabilisers: 0.02% Sodium Azide (NaN3). 0.5% Bovine Serum Albumin

other info2 :

Histology Positive Control Tissue: Human prostate. Buffer Solution: TRIS buffered saline. Antiserum Preparation: Antiserum to human Desmin (CT) was raised by repeated immunisation of goats with highly purified antigen. Purified IgG was prepared by affinity chromatography. Target Species: Human

products description :

Goat anti Human Desmin antibody recognizes an epitope within the C-terminal (CT) region of human Desmin, a type III intermediate filament (IF), localized near the Z line in sacromeres of skeletal, smooth and cardiac muscle tissue. Desmin plays an essential role in the architecture of muscle cells, connecting the Z-disk to the subsarcolemmal cytoskeleton, and the contractile apparatus to the, mitochondria, cell nucleus and post-synaptic areas of motor endplates. Desmin is an early indicator for muscle tissue in embryogenesis, and a marker for tumours of myogenic origin. Mutations in the Desmin gene, are responsible for the neuromuscular disorder known as desmin-related cardio skeletal myopathy (CSM), characterized by skeletal muscle weakness.

ncbi gi num :

55749932

ncbi acc num :

NP_001918.3

ncbi gb acc num :

NM_001927.3

uniprot acc num :

P17661

ncbi mol weight :

53,536 Da

ncbi pathways :

Arrhythmogenic Right Ventricular Cardiomyopathy Pathway (672454); Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway (117293); Arrhythmogenic Right Ventricular Cardiomyopathy (ARVC) Pathway (116129); Aurora B Signaling Pathway (138080); Dilated Cardiomyopathy Pathway (121494); Dilated Cardiomyopathy Pathway (121285); Hypertrophic Cardiomyopathy (HCM) Pathway (114229); Hypertrophic Cardiomyopathy (HCM) Pathway (106591); Muscle Contraction Pathway (106261); Striated Muscle Contraction Pathway (198903)

ncbi summary :

This gene encodes a muscle-specific class III intermediate filament. Homopolymers of this protein form a stable intracytoplasmic filamentous network connecting myofibrils to each other and to the plasma membrane. Mutations in this gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies. [provided by RefSeq, Jul 2008]

uniprot summary :

desmin: a class-III intermediate filaments found in muscle cells. In adult striated muscle they form a fibrous network connecting myofibrils to each other and to the plasma membrane from the periphery of the Z-line structures. Mutations in this gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies. Protein type: Cytoskeletal; Motility/polarity/chemotaxis. Chromosomal Location of Human Ortholog: 2q35. Cellular Component: intermediate filament; fascia adherens; Z disc; cytosol; neuromuscular junction; sarcolemma. Molecular Function: identical protein binding; protein binding; structural constituent of cytoskeleton; cytoskeletal protein binding. Biological Process: muscle contraction; cytoskeleton organization and biogenesis; regulation of heart contraction; muscle filament sliding. Disease: Cardiomyopathy, Dilated, 1i; Scapuloperoneal Syndrome, Neurogenic, Kaeser Type; Myopathy, Myofibrillar, 1; Muscular Dystrophy, Limb-girdle, Type 2r

size1 :

0.1 mg

price1 :

500 USD

more info or order :

MyBioSource product webpage