Validation Data Gallery
|Positive WB detected in||HeLa cells, mouse liver tissue, mouse brain tissue, mouse kidney tissue, mouse heart tissue, mouse skeletal muscle tissue|
|Positive IP detected in||mouse kidney tissue|
|Positive IHC detected in||human kidney tissue|
Note: suggested antigen retrieval with TE buffer pH 9.0; (*) Alternatively, antigen retrieval may be performed with citrate buffer pH 6.0
|Positive IF detected in||HepG2 cells, HeLa cells|
|Western Blot (WB)||WB : 1:1000-1:4000|
|Immunoprecipitation (IP)||IP : 0.5-4.0 ug for IP and 1:500-1:2000 for WB|
|Immunohistochemistry (IHC)||IHC : 1:100-1:400|
|Immunofluorescence (IF)||IF : 1:10-1:100|
|Sample-dependent, check data in validation data gallery|
14631-1-AP targets AFG3L2 in WB, IP, IHC, IF,ELISA applications and shows reactivity with human, mouse, rat samples.
|Tested Reactivity||human, mouse, rat|
|Cited Reactivity||human, mouse, zebrafish|
|Host / Isotype||Rabbit / IgG|
|Immunogen||AFG3L2 fusion protein Ag6209|
|Full Name||AFG3 ATPase family gene 3-like 2 (yeast)|
|Calculated molecular weight||88 kDa|
|Observed molecular weight||80-90 kDa|
|GenBank accession number||BC065016|
|Gene ID (NCBI)||10939|
|Purification Method||Antigen affinity purification|
|Storage Buffer||PBS with 0.02% sodium azide and 50% glycerol pH 7.3.|
|Storage Conditions||Store at -20°C. Stable for one year after shipment. Aliquoting is unnecessary for -20oC storage.|
AFG3L2 is the catalytic subunit of the m-AAA protease, an ATP-dependent proteolytic complex of the mitochondrial inner membrane that degrades misfolded proteins and regulates ribosome assembly(PMID:17101804). Human AFG3L2 is an 80-kDa protein encoded by a 17-exon gene and highly and selectively expressed in human cerebellar Purkinje cells(PMID:20208537) and it can exsit as a truncated 65 kDa protein(PMID:18337413). Defects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28) and spastic ataxia autosomal recessive type 5 (SPAX5).
|Product Specific Protocols|
|WB protocol for AFG3L2 antibody 14631-1-AP||Download protocol|
|IHC protocol for AFG3L2 antibody 14631-1-AP||Download protocol|
|IF protocol for AFG3L2 antibody 14631-1-AP||Download protocol|
|IP protocol for AFG3L2 antibody 14631-1-AP||Download protocol|
|Click here to view our Standard Protocols|
Life Sci Alliance
Mitochondrial stress response triggered by defects in protein synthesis quality control.
Nucleic Acids Res
Deletion of Gtpbp3 in zebrafish revealed the hypertrophic cardiomyopathy manifested by aberrant mitochondrial tRNA metabolism.
Adult-onset obesity is triggered by impaired mitochondrial gene expression.
Clin Sci (Lond)
Novel MTND1 mutations cause isolated exercise intolerance, complex I deficiency and increased assembly factor expression.
Fidelity of translation initiation is required for coordinated respiratory complex assembly.
Ann Clin Transl Neurol
Expanding the clinical and genetic heterogeneity of SPAX5.