AL7A1 rabbit pAb

  • Catalog Number:
    RA19481
  • Size:
    40μL 100μL 200μL
  • Price:
    960 / 2300 / 3780 RMB(含税报价)
  • Form:
    Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
  • Reactivity:
    Human ; Mouse ; Rat
  • Application:
    WB
功能
细胞定位
组织表达
catalytic activity:L-2-aminoadipate 6-semialdehyde + NAD(P)(+) + H(2)O = L-2-aminoadipate + NAD(P)H.,disease:Defects in ALDH7A1 are the cause of pyridoxine-dependent epilepsy (PDE) [MIM:266100]. PDE is characterized by a combination of various seizure types. It usually occurs in the first hours of life and is unresponsive to standard anticonvulsants, responding only to immediate administration of pyridoxine hydrochloride.,similarity:Belongs to the aldehyde dehydrogenase family.,subunit:Homotetramer.,tissue specificity:Abundant in hepatoma cells and fetal cochlea, ovary, eye, heart, adrenal gland, liver and kidney. Low levels present in adult peripheral blood leukocytes and fetal brain, thymus, spleen, skeletal muscle, lung and tongue.,
[Isoform 2]: Cytoplasm, cytosol . Nucleus .; [Isoform 1]: Mitochondrion .
Abundant in hepatoma cells and fetal cochlea, ovary, eye, heart, adrenal gland, liver and kidney. Low levels present in adult peripheral blood leukocytes and fetal brain, thymus, spleen, skeletal muscle, lung and tongue.