Lam R
Department of Pathology, Northwest Community Hospital, Arlington Heights, IL.
Pathol Res Pract. 1990 Oct;186(5):642-50. doi: 10.1016/S0344-0338(11)80228-1.
Enzyme histochemical study revealed that a sacrococcygeal chordoma not only was rich in oxidoreductive enzymes but also in the enzymes (phosphorylase, hexokinase, phosphoglucomutase, glucose phosphate isomerase and UDP-glucose dehydrogenase) leading to the synthesis of stromal glycosaminoglycans from glycogen. UDP-glucose dehydrogenase is particularly important in oxidizing UDP-glucose to UDP-glucuronic acid, the building block of hyaluronic acid and chondroitin sulfates. These enzymatic activities were consistent with the ultrastructural findings of abundant membrane-bound glycogen as well as large intracytoplasmic vacuoles with occasional residual glycogen particles. Furthermore, ultrastructural histochemical study using high iron diamine (HID) specifically localized the sulfated glycosaminoglycans (SG) extracellularly as well as intracellularly in distended Golgi saccules and 187-320 nm mature secretory vesicles. No HID staining was noted in the large intracytoplasmic vacuoles or rough endoplasmic reticulum. This study not only supports the hypothesis that the vacuoles of physaliphorous cells are the result of breakdown and utilization of membrane bound glycogen in the biosynthesis of SG, but also demonstrates that intracellular synthesis and storage of SG in chordoma are not in large vacuoles as previous investigators have believed.
酶组织化学研究显示，骶尾部脊索瘤不仅富含氧化还原酶，还富含能将糖原合成为基质糖胺聚糖的酶（磷酸化酶、己糖激酶、磷酸葡萄糖变位酶、磷酸葡萄糖异构酶和尿苷二磷酸葡萄糖脱氢酶）。尿苷二磷酸葡萄糖脱氢酶在将尿苷二磷酸葡萄糖氧化为尿苷二磷酸葡萄糖醛酸（透明质酸和硫酸软骨素的组成成分）的过程中尤为重要。这些酶活性与超微结构发现一致，即存在丰富的膜结合糖原以及大的胞质内空泡，偶尔还有残留的糖原颗粒。此外，使用高铁二胺（HID）的超微结构组织化学研究明确显示，硫酸化糖胺聚糖（SG）在细胞外以及细胞内的扩张高尔基体囊泡和187 - 320纳米的成熟分泌小泡中均有定位。在大的胞质内空泡或粗面内质网中未观察到HID染色。本研究不仅支持了泡状细胞的空泡是膜结合糖原在SG生物合成中分解和利用的结果这一假说，还表明脊索瘤中SG的细胞内合成和储存并非如先前研究者所认为的那样存在于大空泡中。
