DES Antilichaam - N-terminalgebied: FITC (ARP41482 T100-FITC)

Desmin

CSM1, CSM2, CDCD3, LGMD1D, LGMD1E, LGMD2R

1674

P17661

NP_001918

Human, Mouse, Rat, Cow, Dog, Guinea Pig, Horse, Rabbit

The immunogen is a synthetic peptide directed towards the N terminal region of human DES

DES is 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 its gene are associated with desmin-related myopathy, a familial cardiac and skeletal myopathy (CSM), and with distal myopathies.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.

PPP1R18; UBE2I; EHHADH; TRIM63; TRIM55; UBC; YWHAQ; MAPKAPK2; CAPN1; MLH1; AURKB; HADHB; MYL6; JUP; HSP90AA5P; HNRNPUL1; CCT7; SNRNP70; RPL29; RPL17; UBD; CRYAB; SHBG; SP1; ITSN1; NEB; PLEKHA5; SYNC; TRIM7; SYNM; ROCK1; PKD1; SPTAN1; S100B; S100A1; DSP

FITC: Fluorescein Isothiocyanate

Polyclonal Antibody

WB

0.5 mg/ml

Cow: 100%; Dog: 100%; Guinea Pig: 100%; Horse: 100%; Human: 100%; Mouse: 100%; Rabbit: 100%; Rat: 100%

Liquid. Purified antibody supplied in 1x PBS buffer.

All conjugated antibodies should be stored in light-protected vials or covered with a light protecting material (i.e. aluminum foil) . Conjugated antibodies are stable for at least 12 months at 4C. If longer storage is desired (24 months), conjugates may be diluted with up to 50% glycerol and stored at -20C to -80C. Freezing and thawing conjugated antibodies will compromise enzyme activity as well as antibody binding.

52kDa

Fidzianska, A., (2005) Neuromuscul. Disord. 15 (8), 525-531

Wet Ice

470

DES

DES

Rabbit

Desmin

NM_001927

PLSSPVFPRAGFGSKGSSSSVTSRVYQVSRTSGGAGGLGSLRASRLGTTR