ALAS2 overexpressed transgenic mice exhibited demyelinating injury and muscle atrophy
δ-aminolevulinic acid(ALA)is the first product of the heme synthesis pathway.There are two kinds of ALA synthase(ALAS),ALAS1 and ALAS2,of which ALAS2 is expressed only in erythroid cells.Mice that achieved systemic overexpression of ALAS2 by transgenic technology had obvious amyotrophic phenotypes,when compared with wild-type mice of the same age,the response time of neural reflex experiment was prolonged,the duration of fatigue rotor experiment was shortened at 7 months of age,the spinal cord and medulla oblongata were loosed under transmission electron microscopy at 10 months of age,and denervation-like muscle atrophy was seen by gastrocnemius HE staining.The catalytic product of ALAS2 is neurotoxic,and the damage to the nervous system caused by excess ALA may be one of the causes of muscle atrophy in this transgenic mouse.
万方数据
维普
