ACTN3 Rabbit Polyclonal Antibody

Specifications

Product Data
Applications	IF, WB
Recommend Dilution	Western blot: 1/500-1/1000. Immunofluorescence: 1/50-1/200.
Reactivity	Human, Mouse, Rat
Host	Rabbit
Clonality	Polyclonal
Immunogen	Synthetic peptide, corresponding to the N-terminus of Human Actinin-α3.
Specificity	This antibody detects endogenous levels of Actinin-α3 protein. (region surrounding Glu25)
Formulation	Phosphate buffered saline (PBS), pH 7.2. State: Aff - Purified State: Liquid purified Ig fraction Preservative: 0.05% sodium azide
Concentration	1.0 mg/ml
Purification	Affinity-chromatography using epitope-specific immunogen and the purity is > 95% (by SDS-PAGE)
Conjugation	Unconjugated
Storage Condition	Store undiluted at 2-8°C for one month or (in aliquots) at -20°C for longer. Avoid repeated freezing and thawing.
Predicted Protein Size	~ 103 kDa
Gene Name	actinin alpha 3 (gene/pseudogene)
Database Link	Entrez Gene 89 Human UniProt ID Q08043
Background	The spectrin gene family encodes a diverse group of cytoskeletal proteins that include spectrins, dystrophins and α-actinins. There are four tissue-specific α-actinins, namely α-actinin-1, α-actinin-2, α-actinin-3 and α-actinin-4, which are localized to muscle and non-muscle cells, including skeletal, cardiac and smooth muscle cells, as well as within the cytoskeleton. Each α-actinin protein contains one Actin-binding domain, two calponin-homology domains, two EF-hand domains and four spectrin repeats, through which they function as bundling proteins that can cross-link F-Actin, thus anchoring Actin to a variety of intracellular structures. Defects in the gene encoding α-actinin-4 are the cause of focal segmental glomerulosclerosis 1 (FSGS1), a common renal lesion characterized by decreasing kidney function and, ultimately, renal failure.
Synonyms	Alpha actinin skeletal muscle isoform 3
Reference Data

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