- Catalog number
- A01994-1
- Size / count
- 100 µg/Vial
- Dry ice
- No
- Hazardous
- No
- Discontinued
- No
- Background
- Atlastin, or Atlastin-1, is a protein that in humans is encoded by the ATL1 gene. The protein encoded by this gene is a GTPase and a Golgi body transmembrane protein. The encoded protein can form a homotetramer and has been shown to interact with spastin and with mitogen-activated protein kinase kinase kinase kinase 4. This protein may be involved in axonal maintenance as evidenced by the fact that defects in this gene are a cause of spastic paraplegia type 3. Three transcript variants encoding two different isoforms have been found for this gene.
- Synonyms
- Eukaryotic translation initiation factor 2-alpha kinase 3; PRKR-like endoplasmic reticulum kinase; Pancreatic eIF2-alpha kinase; HsPEK; EIF2AK3; PEK; PERK
- Gene Name
- ATL1
- Gene ID
- 51062
- UniProt
- Q8WXF7
- Host
- Rabbit
- Reactivity
- Human
- Cross Reactivity
- No cross-reactivity with other proteins.
- Immunogen
- A synthetic peptide corresponding to a sequence in the middle region of human SPG3A/ATL1, which shares 93.8% and 100% amino acid (aa) sequence identity with mouse and rat ATL1, respectively.
- Clonality
- Polyclonal
- Tissue Specificity
- Ubiquitous. A high level expression is seen in secretory tissues.
- Applications
- Flow Cytometry, IHC, WB
- Field of Research
- Diabetes, DNA/RNA, Epigenetics and Nuclear Signaling, Metabolism, Obesity, Translation
- Purification
- Immunogen affinity purified.
- Concentration
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Form
- Lyophilized
- Reconstitution
- Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.
- Function
- Metabolic-stress sensing protein kinase that phosphorylates the alpha subunit of eukaryotic translation initiation factor 2 (eIF-2-alpha/EIF2S1) on 'Ser-52' during the unfolded protein response (UPR) and in response to low amino acid availability. Converts phosphorylated eIF-2-alpha/EIF2S1 either in a global protein synthesis inhibitor, leading to a reduced overall utilization of amino acids, or to a translation initiation activator of specific mRNAs, such as the transcriptional activator ATF4, and hence allowing ATF4-mediated reprogramming of amino acid biosynthetic gene expression to alleviate nutrient depletion. Serves as a critical effector of unfolded protein response (UPR) - induced G1 growth arrest due to the loss of cyclin-D1 (CCND1) . Involved in control of mitochondrial morphology and function.
- References & Citations
- 1. Abel, A., Fonknechten, N., Hofer, A., Durr, A., Cruaud, C., Voit, T., Weissenbach, J., Brice, A., Klimpe, S., Auburger, G., Hazan, J. Early onset autosomal dominant spastic paraplegia caused by novel mutations in SPG3A. Neurogenetics 5: 239-243, 2004. 2. Beetz, C., Nygren, A. O. H., Deufel, T., Reid, E. An SPG3A whole gene deletion neither co-segregates with disease nor modifies phenotype in a hereditary spastic paraplegia family with a pathogenic SPG4 mutation. (Letter) Neurogenetics 8: 317-318, 2007. 3. D'Amico, A., Tessa, A., Sabino, A., Bertini, E., Santorelli, F. M., Servidei, S. Incomplete penetrance in an SPG3A-linked family with a new mutation in the atlastin gene. Neurology 62: 2138-2139, 2004.
- Storage Conditions
- At -20°C for one year from date of receipt. After reconstitution, at 4°C for one month. It can also be aliquotted and stored frozen at -20°C for six months. Avoid repeated freezing and thawing.
- Observed Molecular Weight
- 64 kDa
- Applications Notes
- 6
- Gene Name Synonym
- Eukaryotic translation initiation factor 2-alpha kinase 3
- Subcellular Location
- Endoplasmic reticulum membrane; Single-pass type I membrane protein.
- Isotype
- Rabbit IgG
- Contents
- Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.