Mouse anti-Human, Mouse alpha-Actinin 4 (ACTN4) Monoclonal Antibody | anti-ACTN4 antibody

Anti-alpha-Actinin 4 (ACTN4) Antibody

Cat. Num. AAA500081

• Gene Names: ACTN4; FSGS; FSGS1; ACTININ-4
• Reactivity: Human, Mouse
• Applications: Western Blot, Immunohistochemistry
• Purity: Protein G Purified Culture Supernatant
• Synonyms: alpha-Actinin 4 (ACTN4); Monoclonal Antibody; Anti-alpha-Actinin 4 (ACTN4) Antibody; Alpha Actinin 4; anti-ACTN4 antibody

• Host: Mouse
• Reactivity: Human, Mouse
• Clonality: Monoclonal
• Clone Number: 93
• Specificity: Specific for endogenous levels of the ~105 kDa alpha-actinin 4 protein.
• Purity/Purification: Protein G Purified Culture Supernatant
• Form/Format: 100 ul in 10 mM HEPES (pH 7.5), 150 mM NaCl, 100 ug per ml BSA and 50% glycerol.
• Sequence Length: 911

• Applicable Applications for anti-ACTN4 antibody: Western Blot (WB), Immunohistochemistry (IHC)
• Application Notes: WB: 1:1000; IHC: 1:250
• Immunogen Species: Mouse
• Immunogen Information: Fusion protein from the central rod domain of alpha-actinin 4.
• Antigen: Fusion protein from the central rod domain of alpha-actinin 4.
• Quality Control Tests: Western blots performed on each lot.
• Preparation and Storage: Storage at -20 degree C is recommended, as aliquots may be taken without freeze/thawing due to presence of 50% glycerol. Stable for at least 1 year at -20 degree C.

Western Blot (WB)

(Western blot of mouse whole brain lysate showing specific immunolabeling of the ~105 kDa alpha-actinin 4 protein.)

Related Product Information for anti-ACTN4 antibody

alpha-actinin-4 is a member of the actinin protein family comprised of an actin-binding domain in the N-terminus, 4 spectrin-like repeats in the central region, and 2 EF-hand motifs in the C-terminus (Honda et al, 1998). alpha-actinin-4 and CLP36 form a complex in normal kidney podocytes.CLP36 is dependent on alpha-actinin-4 for maintenance of its level in podocytes, whereas alpha-actinin-4 is independent of CLP36. alpha-actinin-4 is widely expressed in mammalian tissues and organs, while having a high occurrence of genetic mutations in kidney podocytes (Kos et al, 2003). FSGS, focal segmental glomerulosclerosis, is a rare genetic disease that attacks the kidney's filtering units (glomeruli) causing serious scarring which leads to permanent kidney damage and even failure. Three key mutations have been found in alpha-actinin-4 in people identified with FSGS. R310Q and Q348R, located in the spectrin-like repeats region, and K255E located in the actin-binding region. The R310Q and Q348R mutation significantly inhibits the ability of alpha-actinin-4 to form the complex with CLP36. The K255E mutation was reversed where it increased the ability to bind CLP36 in the actin-binding region (Liu et al, 2011).

References

1. Liu Z, Blattner SM, Yizeng T, Tisherman R, Wang JH, Rastaldi MP, Kretzler M, Wu C. alpha-Actinin-4 and CLP36 Protein Deficiencies Contribute to Podocyte Defects in Multiple Human Glomerulopathies. J Biol Chem 286(35):30795-805 (2011). 2. Honda K, Yamada T, Endo R, ino Y, Gotoh M, Tsuda H, Yamada Y, Chiba H, Hirohashi S. Actinin-4, a novel actin-bundling protein associated with cell motility and cancer invasion. J Cell Biol. 140(6): 1383-93 (1998). 3. Kos CH, Le TC, Sinha S, Henderson JM, Kim SH, Sugimoto H, Kalluri R, Gerszten RE, Pollak MR. Mice deficient in alpha-actinin-4 have severe glomerular disease. J Clin Invest 111(11):1683-90 (2003).

NCBI and Uniprot Product Information

• NCBI GI #: 13123943
• NCBI GeneID: 81
• UniProt Accession #: O43707
• Molecular Weight: ~105 kDa
• NCBI Official Full Name: Alpha-actinin-4
• NCBI Official Synonym Full Names: actinin alpha 4
• NCBI Official Symbol: ACTN4
• NCBI Official Synonym Symbols: FSGS; FSGS1; ACTININ-4
• NCBI Protein Information: alpha-actinin-4
• UniProt Protein Name: Alpha-actinin-4
• Protein Family: Alpha-actinin
• UniProt Gene Name: ACTN4
• UniProt Entry Name: ACTN4_HUMAN

NCBI Description

Alpha actinins belong to the spectrin gene superfamily which represents a diverse group of cytoskeletal proteins, including the alpha and beta spectrins and dystrophins. Alpha actinin is an actin-binding protein with multiple roles in different cell types. In nonmuscle cells, the cytoskeletal isoform is found along microfilament bundles and adherens-type junctions, where it is involved in binding actin to the membrane. In contrast, skeletal, cardiac, and smooth muscle isoforms are localized to the Z-disc and analogous dense bodies, where they help anchor the myofibrillar actin filaments. This gene encodes a nonmuscle, alpha actinin isoform which is concentrated in the cytoplasm, and thought to be involved in metastatic processes. Mutations in this gene have been associated with focal and segmental glomerulosclerosis. [provided by RefSeq, Jul 2008]

Uniprot Description

ACTN4: F-actin cross-linking protein which is thought to anchor actin to a variety of intracellular structures. This is a bundling protein. Probably involved in vesicular trafficking via its association with the CART complex. The CART complex is necessary for efficient transferrin receptor recycling but not for EGFR degradation. Defects in ACTN4 are the cause of focal segmental glomerulosclerosis type 1 (FSGS1). A renal pathology defined by the presence of segmental sclerosis in glomeruli and resulting in proteinuria, reduced glomerular filtration rate and edema. Renal insufficiency often progresses to end-stage renal disease, a highly morbid state requiring either dialysis therapy or kidney transplantation. Belongs to the alpha-actinin family.

Protein type: Motility/polarity/chemotaxis; Cytoskeletal

Chromosomal Location of Human Ortholog: 19q13

Cellular Component: cortical cytoskeleton; extracellular space; neuron projection; focal adhesion; protein complex; perinuclear region of cytoplasm; cytoplasm; stress fiber; extracellular region; intercellular junction; pseudopodium; Z disc; ribonucleoprotein complex; nucleus

Molecular Function: actin filament binding; integrin binding; protein binding; protein homodimerization activity; ligand-dependent nuclear receptor transcription coactivator activity; retinoic acid receptor binding; nucleoside binding; protein N-terminus binding; calcium ion binding; actin binding; nuclear hormone receptor binding

Biological Process: regulation of apoptosis; retinoic acid receptor signaling pathway; platelet activation; actin filament bundle formation; protein transport; platelet degranulation; response to hypoxia; negative regulation of cell motility; positive regulation of cell motility; positive regulation of sodium:hydrogen antiporter activity; positive regulation of pinocytosis; blood coagulation

Disease: Focal Segmental Glomerulosclerosis 1