ACER3 hydrolyzes phytoceramide

ACER3 (alkaline ceramidase 3) catalyzes the hydrolysis of phytoceramide to yield a free fatty acid and phytosphingosine. The fully functional ACER3 has a Ca2+ and a Zn2+ ion bound (Vasiliauskaité-Brooks et al., 2018). ACER3 hydrolyses ceramides, dihydroceramides, and phytoceramides with long unsaturated acyl chains (Hu et al., 2010; Casasampere et al., 2015). ACER3 mRNA is widely expressed in the body, although most abundant in the placenta. Immunofluorescence studies of cultured cells over-expressing GFP-tagged protein suggest its localization to membranes of the endoplasmic reticulum (annotated here) and the Golgi apparatus (Mao et al. 2001). Deficiencies in ACER3 activity lead to a form of leukodystrophy (PLDECO, MIM:617762) (reviewed by Mao & Obeid, 2008; Parveen et al., 2019; Duarte et al., 2020; Dehnavi et al., 2021).

ACER3（碱性鞘氨醇酶3）催化植物鞘氨醇的水解，生成游离脂肪酸和植物鞘氨醇。功能完备的ACER3与Ca2+和Zn2+离子结合（Vasiliauskaité-Brooks等，2018年）。ACER3可水解鞘氨醇、二氢鞘氨醇和长链不饱和酰基链的植物鞘氨醇（Hu等，2010年；Casasampere等，2015年）。ACER3 mRNA在全身广泛表达，尽管在胎盘中含量最为丰富。对过表达GFP标记蛋白的培养细胞进行的免疫荧光研究表明，其定位于内质网（此处标注）和高尔基体膜（Mao等，2001年）。ACER3活性缺乏会导致一种形式的白质营养不良（PLDECO，MIM：617762）（Mao & Obeid，2008年；Parveen等，2019年；Duarte等，2020年；Dehnavi等，2021年综述）。