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AFG3-like protein 2 Antibodies
AND AFG3L2 ELISA kits, AFG3-like protein 2 Proteins
Many biological assays use antibodies to detect AFG3-like protein 2, such as Western Blot (WB), ELISA, Flow Cytometry, Immunocytochemistry (IHC). These antibodies can be polyclonal or monoclonal, and react with AFG3-like protein 2 in Human, Mouse, Rat, and many other animal samples. Boster Bio use mainly mouse and rabbit to develop AFG3-like protein 2 antibodies...
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AFG3L2 Infographic by Boster Bio
All AFG3L2 Products
Product
Figures
Specificity
Applications
Price
Human, Mouse, Rat
Flow Cytometry, IP, IF, ICC, WB
$370
Cat # PB9915
100 μg/vial
Cat # PB9915
100 μg/vial
Human, Mouse, Rat
WB
$523
Cat # M04820
100 µl
Cat # M04820
100 µl
AFG3L2 Overview
Facts about AFG3-like protein 2.
AFG3-like protein 2 (AFG3L2)
ATP-dependent protease which is essential for axonal and neuron development. In neurons, mediates degradation of SMDT1/EMRE before its assembly with the uniporter complicated, limiting the access to SMDT1/EMRE for MCU assembly and encouraging efficient assembly of gatekeeper subunits with MCU (PubMed:27642048).
Required for the maturation of paraplegin (SPG7) after its cleavage by mitochondrial-processing peptidase (MPP), converting it into a proteolytically active mature form (By similarity). .
Gene Information
| Human | |
|---|---|
| Gene Name: | AFG3L2 |
| Uniprot: | Q9Y4W6 |
| Entrez: | 10939 |
Family
| Belongs to: |
|---|
| No superfamily |
Alternative Names
AFG3 (ATPase family gene 3, yeast)-like 2; AFG3 ATPase family gene 3-like 2 (S. cerevisiae); AFG3 ATPase family gene 3-like 2 (yeast); AFG3-like protein 2; ATPase family gene 3, yeast; EC 3.4.24; EC 3.4.24.-; FLJ25993; Paraplegin-like protein; SCA28; spinocerebellar ataxia 28
Molecular Weight
Mass (kDA):
88.584 kDA
Genomic Context
| Human | |
|---|---|
| Location: | 18p11.21 |
| Sequence: | 18; NC_000018.10 (12328944..12377309, complement) |
Tissue Specificity
Ubiquitous. Highly expressed in the cerebellar Purkinje cells.
Subcellular Localizations
Mitochondrion. Mitochondrion inner membrane; Multi-pass membrane protein.
Diseases
- Ataxia
- Neurodegenerative Disorders
- Ataxia, Spinocerebellar
- Paraplegia
- Spastic Paraplegia
- Spastic Paraplegia, Hereditary
- Aortic Aneurysm, Abdominal
- Cerebellar Diseases
- Nerve Degeneration
- Cerebellar Ataxia
- Spinocerebellar Degeneration
- Atrophy
Pathways
- Pathogenesis
- Mitochondrial Fusion
- Ribosome Assembly
- Localization
- Translation
- Cell Death
- Mitochondrial Unfolded Protein Response
- Proteolysis
PTMs
- Cleavage
For details, visit:
bosterbio.com/bosterbio-gene-info-cards/AFG3L2
bosterbio.com/bosterbio-gene-info-cards/AFG3L2
References
PMID: 10395799 by Banfi S., et al. Identification and characterization of AFG3L2, a novel paraplegin- related gene.
PMID: 14623864 by Atorino L., et al. Loss of m-AAA protease in mitochondria causes complex I deficiency and increased sensitivity to oxidative stress in hereditary spastic paraplegia.