Dysferlin (DYSF) Rabbit Polyclonal Antibody

About this product

The most intensively studied role for dysferlin is in a cellular process called membrane repair. Membrane repair is a critical mechanism by which cells are able to seal dramatic wounds to the plasma membrane. Muscle is thought to be particularly prone to membrane wounds given that muscle cells transmit high force and undergo cycles of contraction. Dysferlin is highly expressed in muscle, and is homologous to the ferlin family of proteins, which are thought to regulate membrane fusion across a wide variety of species and cell types. Several lines of evidence suggest that dysferlin may be involved in membrane repair in muscle. First, dysferlin-deficient muscle fibers show accumulation of vesicles (which are critical for membrane repair in non-muscle cell types) near membrane lesions, indicating that dysferlin may be required for fusion of repair vesicles with the plasma membrane. Further, dysferlin-deficient muscle fibers take up extracellular dyes to a greater extent than wild-type muscle fibers following laser-induced wounding in-vitro.

Specifications

Catalog number

ER1511-12

Size / count

100 µL

Dry ice

No

Hazardous

No

Discontinued

No

CAS Number

9007-83-4

Product Name Alternative

DMAT antibody DYSF antibody DYSF_HUMAN antibody Dysferlin antibody Dysferlin limb girdle muscular dystrophy 2B (autosomal recessive) antibody Dysferlin limb girdle muscular dystrophy 2B antibody Dystrophy associated fer 1 like 1 antibody Dystrophy associated fer 1 like protein antibody Dystrophy associated fer1 like 1 antibody Dystrophy associated fer1 like protein antibody Dystrophy-associated fer-1-like protein antibody Fer 1 like protein 1 antibody Fer-1-like protein 1 antibody Fer1 like protein 1 antibody FER1L1 antibody FLJ00175 antibody FLJ90168 antibody LGMD 2B antibody LGMD2B antibody Limb girdle muscular dystrophy 2B (autosomal recessive) antibody Limb girdle muscular dystrophy 2B antibody Miyoshi myopathy antibody MM antibody MMD1 antibody

Abbreviation

DYSF, FER1L1

Swiss Prot

O75923 Human

Cellular Locus

Plasma membrane, Cytoplasmic vesicle.

Host

Rabbit

Species Reactivity

Human

Immunogen

Recombinant protein within Human Dysferlin aa 124-317 / 2,080.

Isotype

IgG

Conjugation

Non-conjugated

Type

Rabbit polyclonal Antibody

Applications

WB, IHC-P, FC

Positive Control

Human fetal skeletal muscle tissue lysate, human kidney tissue, human placenta tissue, human fetal skeletal muscle tissue, HUVEC.

Concentration

1 mg/mL

Dilution

WB: 1:500-1:2,000 ;IHC-P: 1:50-1:200 ;FC: 1:50-1:100

Purity

Immunogen affinity purified.

Form

Liquid

Buffer

1*PBS (pH7.4), 0.2% BSA, 50% Glycerol. Preservative: 0.05% Sodium Azide.

Molecular Weight

Predicted band size: 237 kDa

Storage Conditions

Store at +4°C after thawing. Aliquot store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

Recombinant Antibody

No