Anti-Dysferlin (N-terminal) antibody produced in rabbit SAB4200453-200UL

NACRES:

NA.41

Dysferlin is a 230 kDa transmembrane protein that belongs to the ferlin family of proteins including myoferlin and otoferlin and is homologous to the C. elegans fer-1 protein. It is expressed early during human development.
synthetic peptide corresponding to a sequence in the N-terminal region of human dysferlin, conjugated to KLH. The corresponding sequence is identical in human dysferlin isoforms 1-14 and identical in mouse dysferlin.
Anti-Dysferlin (N-terminal) antibody produced in rabbit has been used in several immunochemical techniques including immunoblotting, immunofluorescence and immunohistochemistry.
Biochem/physiol Actions
Dysferlin is implicated in membrane fusion events. It has also been involved in membrane repair processes, such as the ability to reseal the sarcolemma upon muscle injury. The integral membrane protein caveolin 3 have been shown to regulate the endocytosis of dysferlin. Dysferlin localization in the membrane and trafficking is impaired by mutations in caveolin-1 and 3, resulting in mistargeting and redistribution of dysferlin from the plasma membrane to the Golgi complex. Mutations in the dysferlin gene leads to the development of limb-girdle muscle dystrophy type 2B (LGMD2B), an autosomal recessive disorder and the related Miyoshi myopathy.
Physical form
Solution in 0.01 M phos­phate buffered saline, pH 7.4, containing 15 mM sodium azide.
Disclaimer
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.

biological source	rabbit
Quality Level	200
antibody form	affinity isolated antibody
antibody product type	primary antibodies
clone	polyclonal
form	buffered aqueous solution
mol wt	antigen ~250 kDa
species reactivity	rat, human, mouse
enhanced validation	recombinant expression
concentration	~1.0 mg/mL
application(s)	dot blot: 0.1-0.2 µg/mL using extracts of HEK-293T cells over-expressing human dysferlin and 1-2 mg/mL using A10 cells.","immunohistochemistry: 20-30 µg/mL using methanol-acetone fixed frozen sections of mouse skeletal muscle.","indirect immunofluorescence: 5-10 µg/mL using differentiated C2C12 myoblasts.
conjugate	unconjugated
UniProt accession no.	O75923,
shipped in	dry ice
storage temp.	20°C
Gene Information	human ... DYSF(8291) mouse ... Dysf(26903) rat ... Dysf(312492)

RIDADR	NONH for all modes of transport
Flash Point F	Not applicable
Flash Point C	Not applicable