Mouse anti-Human Alpha Actinin 4/ACTN4 Monoclonal Antibody | anti-ACTN4 antibody

Alpha Actinin 4/ACTN4

Cat. Num. AAA4381356

• Gene Names: ACTN4; FSGS; FSGS1; ACTININ-4
• Reactivity: Human
• Applications: Immunohistochemistry
• Purity: Purified Ab with BSA and Azide at 200ug/ml OR Purified Ab WITHOUT BSA and Azide at 1.0mg/ml
• Synonyms: Alpha Actinin 4/ACTN4; Monoclonal Antibody; F-actin cross-linking protein; Focal segmental glomerulosclerosis 1 (FSG1); Non-muscle alpha-actinin-4; anti-ACTN4 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Monoclonal
• Isotype: IgG
• Clone Number: 93
• Specificity: The spectrin gene family encodes a diverse group of cytoskeletal proteins that include spectrins, dystrophins and -actinins. There are four tissue-specific -actinins, namely -actinin-1, -actinin-2, -actinin-3 and -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 -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 -actinin-4 are the cause of focal segmental glomerulosclerosis 1 (FSGS1), a common renal lesion characterized by decreasing kidney function and, ultimately, renal failure.
• Purity/Purification: Purified Ab with BSA and Azide at 200ug/ml OR Purified Ab WITHOUT BSA and Azide at 1.0mg/ml
• Form/Format: 200ug/ml of Ab Purified from Bioreactor Concentrate by Protein A/G. Prepared in 10mM PBS with 0.05% BSA & 0.05% azide. Also available WITHOUT BSA & azide at 1.0mg/ml.
• Sequence Length: 911

• Applicable Applications for anti-ACTN4 antibody: Immunohistochemistry (IHC) Paraffin
• Application Notes: Immunohistochemistry (Formalin-fixed) (1-2ug/ml for 30 minutes at RT) (Staining of formalin-fixed tissues requires boiling tissue sections in 10mM Citrate Buffer, pH 6.0, for 10-20 min followed by cooling at RT for 20 minutes)
• Positive Control: Human colon or kidney tissue (IHC).
• Immunogen: Recombinant full-length human ACTN4 protein
• Cellular Localization: Nucleus. Cytoplasm.
• Hu-chromosome Location: 19q13.2
• Preparation and Storage: Antibody with azide: store at 2 to 8 degree C.; Antibody without azide: store at -20 to -80 degree C.; Antibody is stable for 24 months. Non-hazardous. No MSDS required.

SDS-Page

(SDS-PAGE Analysis Purified ACTN4 Mouse Monoclonal Antibody (93). Confirmation of Purity and Integrity of Antibody.)

References

Krusius, T. and Ruoslahti, E. 1986. Proc. Natl. Acad. Sci. USA 83: 7683-7687.

NCBI and Uniprot Product Information

• NCBI GI #: 13123943
• NCBI GeneID: 81
• UniProt Accession #: O43707
• Molecular Weight: 105kDa
• 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