The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
Use a concentration of 3 - 5 µg/ml.
功能May act as a GTPase regulator. Controls survival and growth of spinal motoneurons.
疾病相关Defects in ALS2 are the cause of amyotrophic lateral sclerosis type 2 (ALS2) [MIM:205100]. ALS2 is a familial form of amyotrophic lateral sclerosis, a neurodegenerative disorder affecting upper and lower motor neurons and resulting in fatal paralysis. Sensory abnormalities are absent. Death usually occurs within 2 to 5 years. The etiology of amyotrophic lateral sclerosis is likely to be multifactorial, involving both genetic and environmental factors. The disease is inherited in 5-10% of cases leading to familial forms. Defects in ALS2 are the cause of juvenile primary lateral sclerosis (JPLS) [MIM:606353]. JPLS is a neurodegenerative disorder which is closely related to but clinically distinct from amyotrophic lateral sclerosis. It is a progressive paralytic disorder which results from dysfunction of the upper motor neurons of the motor cortex while the lower neurons are unaffected. Defects in ALS2 are the cause of infantile-onset ascending spastic paralysis (IAHSP) [MIM:607225]. IAHSP is characterized by progressive spasticity and weakness of limbs.
ab4155 at 3.8µg/ml staining Als2 in human cortex tissue section by Immunohistochemistry (Formalin/ PFA-fixed paraffin embedded tissue sections). The tissue underwent antigen retrieval by steam in citrate buffer at pH 6.0. The AP-staining procedure was used for detection.
Anti-Als2 antibody (ab4155)参考文献
has not yet been referenced specifically in any publications.