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Anti-AFG3L2 Rabbit pAb (100 μl)
| Reactivity: | H,M,R |
| Applications: | WB |
| Host Species: | Rabbit |
| Clonality: | Polyclonal |
| Full Name: | AFG3L2 rabbit polyclonal antibody |
Gene Name: | AFG3-like protein 2 |
Synonyms: | AFG3 like protein 2, AFG3L2, Paraplegin like protein, SCA28, Spinocerebellar ataxia 28, AFG3 ATPase family gene 3 like 2 |
Immunogen: | KLH conjugated Synthetic peptide corresponding to Mouse AFG3L2 |
Isotype: | IgG |
Purity: | Affinity purification |
Predicted MW. | 90 kDa |
Observed MW. | 90 kDa |
Uniprot ID: |
Product Usage Information
Applications | Species | Dilution | Positive Sample |
WB | Human, Mouse, Rat | 1: 1000-1: 2000 | liver, brain, kidney |
Background
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. Human AFG3L2 is an 80-kDa protein encoded by a 17-exon gene and highly and selectively expressed in human cerebellar Purkinje cells and it can exsit as a truncated 65 kDa protein. Defects in AFG3L2 are the cause of spinocerebellar ataxia type 28 (SCA28) and spastic ataxia autosomal recessive type 5 (SPAX5).
Images
 | Western blot analysis of AFG3L2 (GB113859) at dilution of 1: 2000 |
Storage
| Storage | Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| Storage Buffer | PBS with 0.02%sodium azide,100 μg/ml BSA and 50% glycerol. |
