α-Actinin 4 (ACTN4) Antibody

Catalog #: 127-ACTN4 Category: Datasheet:

$109.00$365.00

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Mouse monoclonal antibody

Formulation:
Protein G purified
Specificity:
Human, Mouse
Applications:
WB 1:1000IHC 1:250
Species:
Mouse Monoclonal
Gene Name:
ACTN4
Molecular Reference:
~105 kDa
Cite This Antibody:
PhosphoSolutions Cat# 127-ACTN4, RRID:AB_2736847
Antigen/Purification: ExpandCollapse

The antigen is a fusion protein containing the central rod domain of human α-actinin 4.

The antibody is protein G purified from culture supernatant.

Biological Significance: ExpandCollapse

α-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). α-actinin-4 and CLP36 form a complex in normal kidney podocytes.CLP36 is dependent on α-actinin-4 for maintenance of its level in podocytes, whereas α-actinin-4 is independent of CLP36. α-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 α-actinin-4 in people diagnosed 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 α-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).

Storage

100 µl in 10mM HEPES (pH7.5), 150mM NaCl, 100ug BSA per ml and 50% glycerol.

For long term storage –20° C is recommended in undiluted aliquots. Stable at –20° C for at least 1 year. Avoid freeze/thaw cycles.

Product Specific References:

Liu Z, Blattner SM, Yizeng T, Tisherman R, Wang JH, Rastaldi MP, Kretzler M, Wu C. α-Actinin-4 and CLP36 Protein Deficiencies Contribute to Podocyte Defects in Multiple Human Glomerulopathies. J Biol Chem 286(35):30795-805 (2011).

Western blot of Actinin 4 ACTN4 Antibody
Western blot of mouse whole brain lysate showing specific immunolabeling of the ~105 kDa α-actinin 4 protein.

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