Product Name
ATL1, Blocking Peptide
Product Synonym Names
Atlastin-1; 365-; Brain-specific GTP-binding protein; GTP-binding protein 3; GBP-3; hGBP3; Guanine nucleotide-binding protein 3; Spastic paraplegia 3 protein A; ATL1; GBP3; SPG3A
Product Gene Name
ATL1 blocking peptide
[Similar Products]
Product Synonym Gene Name
GBP3; SPG3A[Similar Products]
Antibody/Peptide Pairs
ATL1 peptide (MBS9222013) is used for blocking the activity of ATL1 antibody (MBS9202748)
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for Q8WXF7
Form/Format
Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.
Cellular Location
Endoplasmic reticulum membrane; Multi-pass membrane protein. Golgi apparatus membrane; Multi-pass membrane protein. Cell projection, axon
Tissue Location
Expressed predominantly in the ***** and fetal central nervous system. Measurable expression in all tissues examined, although expression in ***** brain is at least 50-fold higher than in other tissues. Detected predominantly in pyramidal neurons in the cerebral cortex and the hippocampus of the brain Expressed in upper and lower motor neurons (at protein level)
Preparation and Storage
Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C.
Other Notes
Small volumes of ATL1 blocking peptide vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
ATL1 blocking peptide
GTPase tethering membranes through formation of trans- homooligomers and mediating homotypic fusion of endoplasmic reticulum membranes. Functions in endoplasmic reticulum tubular network biogenesis. May also regulate Golgi biogenesis. May regulate axonal development.
NCBI/Uniprot data below describe general gene information for ATL1. It may not necessarily be applicable to this product.
NCBI Accession #
Q8WXF7.1
[Other Products]
UniProt Primary Accession #
Q8WXF7
[Other Products]
UniProt Secondary Accession #
O95890; Q69YH7; Q96FK0; A6NND5; A8K2C0; G5E9T1[Other Products]
UniProt Related Accession #
Q8WXF7[Other Products]
Molecular Weight
63,055 Da
NCBI Official Full Name
Atlastin-1
NCBI Official Synonym Full Names
atlastin GTPase 1
NCBI Official Symbol
ATL1 [Similar Products]
NCBI Official Synonym Symbols
FSP1; GBP3; SPG3; HSN1D; SPG3A; AD-FSP; atlastin1
[Similar Products]
NCBI Protein Information
atlastin-1
UniProt Protein Name
Atlastin-1
UniProt Synonym Protein Names
Brain-specific GTP-binding protein; GTP-binding protein 3; GBP-3; hGBP3; Guanine nucleotide-binding protein 3; Spastic paraplegia 3 protein A
UniProt Gene Name
ATL1 [Similar Products]
UniProt Synonym Gene Names
GBP3; SPG3A; GBP-3; hGBP3 [Similar Products]
UniProt Entry Name
ATLA1_HUMAN
NCBI Summary for ATL1
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. [provided by RefSeq, Jul 2008]
UniProt Comments for ATL1
atlastin: GTPase tethering membranes through formation of trans- homooligomer and mediating homotypic fusion of endoplasmic reticulum membranes. Functions in endoplasmic reticulum tubular network biogenesis. May also regulate Golgi biogenesis. May regulate axonal development. Defects in ATL1 are the cause of spastic paraplegia autosomal dominant type 3 (SPG3); also known as Strumpell-Lorrain syndrome. Spastic paraplegia is a degenerative spinal cord disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Defects in ATL1 are the cause of hereditary sensory neuropathy type 1D (HSN1D). HSN1D is a disease characterized by *****-onset distal axonal sensory neuropathy leading to mutilating ulcerations as well as hyporeflexia. Some patients may show features suggesting upper neuron involvement. Belongs to the GBP family. Atlastin subfamily.
Protein type: EC 3.6.5.-; Membrane protein, multi-pass; Vesicle; Membrane protein, integral
Chromosomal Location of Human Ortholog: 14q22.1
Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane; Golgi apparatus; Golgi cis cisterna; integral to membrane
Molecular Function: GTP binding; GTPase activity; identical protein binding; protein binding
Biological Process: axonogenesis; endoplasmic reticulum organization and biogenesis; protein homooligomerization
Disease: Neuropathy, Hereditary Sensory, Type Id; Spastic Paraplegia 3, Autosomal Dominant
Research Articles on ATL1
1. Novel splicing pathogenic variants were identified in ATL1 genes of Korean patients with hereditary spastic paraplegia.
Precautions
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Disclaimer
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