AFG3L2 is a multi-pass membrane metalloprotease that contains one AAA (ATPase associated with diverse cellular activities) domain, a zinc-dependent binding motif, an RNA-binding region and an ATP/GTP binding site. Localizing to the mitochondrial membrane, AFG3L2 is ubiquitously expressed with highest expression levels in skeletal muscle and heart. AFG3L2 shares 69% similarity with the yeast Afg3 protein and 49% similarity with Paraplegin, a protein of mitochondria that is thought to be involved in signal transduction and chaperone-like activities. In mitochondria, AFG3L2 forms a complex with Paraplegin that is believed to regulate essential protein quality control. Mutations in the gene encoding either one of these proteins can result in hereditary spastic paraplegia, a degenerative spinal cord disorder that is characterized by muscle spasms, stiffness in the legs and, in some cases, incontinence.

SCA28; AFG3 (ATPase family gene 3, yeast) like 2; AFG3 ATPase family gene 3 like 2 (yeast); AFG3 ATPase family gene 3 like 2; AFG3 like protein 2; ATPase family gene 3 like 2; ATPase family gene 3 yeast; FLJ25993; Paraplegin like protein; SCA28; Spinocerebellar ataxia 28; AFG32_HUMAN .

Mouse,Rat,Human,Chicken,Dog,Pig,Cow,Horse,Sheep

IHC,IF,ICC

IHC 1:100-500
IF 1:100-500
ICC 1:100-1:400

89kD

10939

Q9Y4W6

https://www.uniprot.org/uniprotkb/Q9Y4W6

Mitochondrial membrane; multipass membrane protein

Ubiquitous. Highly expressed in the cerebellar Purkinje cells.

KLH conjugated synthetic peptide derived from human AFG3L2

Rabbit

Polyclonal

IgG

affinity purified by Protein A

Liquid

0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.