Anti-ATL1 antibody (AA34066)

$187.00


Alternative names Atlastin-1, EC 3.6.5.-, 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, ATLA1_HUMAN
Species Reactivity Human, Mouse, Rat
Applications IF/ICC, IHC, WB
Host Rabbit
Class Polyclonal
Conjugated Unconjugated


Description

Product name Anti-ATL1 antibody (AA34066)
Catalog number AA34066
Brand family Ango
Product type Primary Antibody
Antibody Alternative Names Atlastin-1 antibody, EC 3.6.5.- antibody, Brain-specific GTP-binding protein antibody, GTP-binding protein 3 antibody, GBP-3 antibody, hGBP3 antibody, Guanine nucleotide-binding protein 3 antibody, Spastic paraplegia 3 protein A antibody, ATL1 antibody, GBP3 antibody, SPG3A antibody, ATLA1_HUMAN antibody


Biological Information

Immunogen Recombinant protein of human ATL1
Concentration 1 mg/ml
Isotype IgG
Application Notes WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200
Purification method Purified by affinity chromatography


Target

Target Name ATL1
SwissProt ID Link Q8WXF7
Function 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.
Subcellular Location Endoplasmic reticulum membrane; Multi-pass membrane protein. Golgi apparatus membrane; Multi-pass membrane protein. Cell projection, axon.
Involvement in Disease Spastic paraplegia 3, autosomal dominant: A form of spastic paraplegia, a neurodegenerative disorder characterized by a slow, gradual, progressive weakness and spasticity of the lower limbs. Rate of progression and the severity of symptoms are quite variable. Initial symptoms may include difficulty with balance, weakness and stiffness in the legs, muscle spasms, and dragging the toes when walking. In some forms of the disorder, bladder symptoms: A disease characterized by adult-onset distal axonal sensory neuropathy leading to mutilating ulcerations as well as hyporeflexia. Some patients may show features suggesting upper neuron involvement. The disease is caused by mutations affecting the gene represented in this entry.
Tissue Specificity Expressed predominantly in the adult and fetal central nervous system. Measurable expression in all tissues examined, although expression in adult 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).
Subunit Structure Monomer as apoprotein and in the GDP-bound form. Homodimer in the GTP-bound form. Interacts (via N-terminal region) with MAP4K4 (via CNH regulatory domain). Interacts with REEP5, RTN3 and RTN4 (via the transmembrane region). Interacts with SPAST; interaction is direct. May interact with TMED2. Interacts with REEP1.
Sequence Similarities Belongs to the TRAFAC class dynamin-like GTPase superfamily. GB1/RHD3-type GTPase family. GB1 subfamily. Contains 1 GB1/RHD3-type G (guanine nucleotide-binding) domain.


Poduct Presentation

Form Liquid
Preservative 0.02% (w/v) sodium azide
Storage buffer PBS with 50% glycerol, pH 7.3


Storage and Shipping Information

Storage Store at -20 or -80 C. Avoid multiple freeze/thaw cycles.