The antibody detects endogenous level of total ATL1 protein.
Applications:
WB, IHC, IF
Reactivity:
Human
Immunogen:
Recombinant protein of human ATL1.
Host:
Rabbit
Clonality:
Polyclonal
Conjugate:
Unconjugated
Purification:
Antibodies were purified by affinity purification using immunogen.
Concentration:
1.0mg / ml
Formulation:
Supplied at 1.0mg / mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
Storage:
Store at -20?C
Target
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.
Tissue Specificity:
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).
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 (such as incontinence) may appear, or the weakness and stiffness may spread to other parts of the body.
Neuropathy, hereditary sensory, 1D: 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.
Sequence Similarities:
Belongs to the TRAFAC class dynamin-like GTPase superfamily. GB1/RHD3-type GTPase family. GB1 subfamily.