Applications
-
WB
-
IHC-P
-
FC
REACTIVITY
-
Human
SPECIFICATIONS
Product Type
Rabbit polyclonal primary
Product Name
Dysferlin (DYSF) Antibody (ER1511-12)
Immunogen
Recombinant protein
Host
Rabbit
Positive Control
Human fetal skeletal muscle tissue, human kidney tissue, human placenta tissue, HUVEC.
Conjugation
Unconjugated
Clonality
Polyclonal
PROPERTIES
Form
Liquid
Storage Condition
Store at +4C after thawing. Aliquot store at -20C or -80C. Avoid repeated freeze / thaw cycles.
Storage Buffer
1*PBS (pH7.4), 0.2% BSA, 50% Glycerol. Preservative: 0.05% Sodium Azide.
Concentration
1 ug/ul
PURIFICATION
Protein affinity purified
MOLECULAR WEIGHT
237 kDa
Isotype
IgG
APPLICATION DILUTION
-
WB
-
1:500-1:2,000
-
IHC-P
-
1:50-1:200
-
FC
-
1:50-1:100
TARGET
UNIPROT #
PROTEIN NAME
Dysferlin (DYSF)
SYNONYMS
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
SEQUENCE SIMILARITIES
Belongs to the ferlin family.
TISSUE SPECIFICITY
Expressed in skeletal muscle, myoblast, myotube and in the syncytiotrophoblast (STB) of the placenta (at protein level). Ubiquitous. Highly expressed in skeletal muscle. Also found in heart, brain, spleen, intestine, placenta and at lower levels in liver, lung, kidney and pancreas.
DEVELOPMENTAL STAGE
Expression in limb tissue from 5-6 weeks embryos; persists throughout development.
SUBCELLULAR LOCATION
Plasma membrane. Cytoplasmic vesicle.
FUNCTION
Key calcium ion sensor involved in the Ca2+-triggered synaptic vesicle-plasma membrane fusion. Plays a role in the sarcolemma repair mechanism of both skeletal muscle and cardiomyocytes that permits rapid resealing of membranes disrupted by mechanical stress.