Metachromatic leukodystrophy caused by a partial cerebroside sulfatase defect

A patient with neuropathy and myopathy since infancy but whose neuropathy had been stable for a number of years showed a profound deficiency of arylsulfatase A in leukocytes and urine. Urine contained material that stained metachromatically and cochromatographed with cerebroside sulfate. In contrast, cultured fibroblasts contained about 10-20% of normal arylsulfatase A with properties identical to properties of normal fibroblast enzyme, except that it showed no cerebroside sulfatase activity. Growing fibroblasts in the cerebroside sulfate loading test had an attenuated rate of sulfatide hydrolysis. A re-examination of the cerebroside sulfatase reaction revealed that while only limited hydrolysis occurred with low concentrations of taurodeoxycholate or cholate (type I activation), significant hydrolysis of the natural substrate did take place with high concentrations of cholate (type II activation). This suggests that there is a partial cerebroside sulfatase defect in this atypical form of metachromatic leukodystrophy.     

Sphingomyelin changes in rat cerebral cortex during focal ischemia

Abstract

To better define the sphingolipid metabolism during focal brain ischemia, levels of ceramide, sphingomyelin, cerebroside and gangliosides were determined in rat cerebral cortex during focal ischemia produced by occlusion of the middle cerebral artery. Sphingomyelin began to decrease at 2 hours of ischemia and continued to decrease for 96 hours. In contrast, ceramide increased at 6 hours and increased to 4.2-fold at 96 hours after ischemia, and the fatty acid composition of ceramide was solely nonhydroxylated fatty acid similar to sphingomyelin. Hydroxylated fatty acid-linked cerebroside decreased at 6 hours of ischemia, whereas any significant decrease of nonhydroxylated fatty acid-linked cerebroside didn't occur for 96 hours of ischemia. There were no measurable changes in the levels of gangliosides. These results suggested that ceramide was produced in the cerebral cortex by the breakdown of sphingomyelin during early ischemia. [Neurol Res 1996; 18: 337-341]     

脑苷肌肽联合川芎嗪注射液治疗脑栓塞的临床研究

目的:观察脑苷肌肽联合川芎嗪注射液治疗脑栓塞的临床疗效。方法:治疗组48例脑栓塞患者应用脑苷肌肽注射液20ml、川芎嗪注射液160mg静滴,每日1次,14d为1个疗程。另选48例作为对照组,采用胞二磷胆碱注射液、川芎嗪注射液治疗。治疗前后进行神经功能缺损评分和生活能力状态级别评分,并进行临床疗效评定。结果:治疗组神经功能缺损评分显著减少(P<0.01),病残程度显著改善(P<0.01),治疗组显效率79.16%,高于对照组58.33%(P<0.05)。结论:脑苷肌肽联合川芎嗪注射液能明显降低脑栓塞患者神经功能缺损程度,提高患者的生活质量,具有可靠的疗效及安全性。     

海地瓜脑苷脂的气相色谱-质谱法联用分析

目的:测定海地瓜脑苷脂组成。方法:样品经10%盐酸-甲醇溶液水解,采用气相色谱-质谱(GC-MS)联用技术对已分离纯化的海地瓜脑苷脂样品进行分析测定。结果:分别分析测定了3种海地瓜脑苷脂(AMC-1、AMC-2、AMC-3)的脂肪酸、长链碱和糖基组成:脂肪酸主要为饱和的、单不饱和的和含羟基的脂肪酸,长链碱有二羟基和三羟基2种结构,糖基主要为葡萄糖。结论:通过GC-MS法分析海地瓜脑苷脂,为脑苷脂类化合物的定性分析和进一步结构研究提供了一定的依据。     

紫芝中的一个新三萜

摘 要：目的 研究紫芝Ganoderma sinense子实体的化学成分。 方法 通过反复硅胶、凝胶柱色谱进行分离纯化,利用多种波谱技术鉴定化合物的结构。结果 分离鉴定了4个化合物,分别为紫芝酸(sinensoic acid, 1)、cerebroside D(2)、poke-weed cerebroside(3)、泥湖鞘鞍醇(hemisceramide,4)。结论 化合物1为新化合物,命名为紫芝酸(sinensoic acid),其余化合物均为首次从灵芝属真菌中分离得到。     

转基因西洋参冠瘿组织化学成分研究(Ⅱ)

目的研究转基因西洋参Panax quinquefolium冠瘿组织中的化学成分。方法采用大孔吸附树脂、硅胶柱等色谱技术对西洋参冠瘿组织乙醇提取物进行分离纯化,并根据理化性质及波谱数据对分离所得的化合物进行结构鉴定。结果从西洋参冠瘿组织乙醇提取物中分离得到10个化合物,分别鉴定为:尿嘧啶核苷(1)、α-D-呋喃果糖甲苷(2)、1-O-β-D-吡喃葡萄糖-(2S,3S4,R,8E)-2-[(2′R)-2′-羟基棕榈酰胺]-8-十八烯-1,3,4-三醇(3,脑苷脂)、豆甾醇(4)、奎酸(5)、奎酸乙酯(6)、十三碳-6-烯(7)、二十四烷醇(8)、邻苯二甲酸二异辛酯(9)、十六酸甲酯(10)。结论化合物3为首次从人参属植物中分离得到,10个化合物均为首次从转基因西洋参冠瘿组织中分离得到。     

Sulphatide-binding properties are shared by serum amyloid P component and a polyreactive germ-line IgM autoantibody,the TH3 idiotype

Serum amyloid P component (SAP) concentration was elevated in sera from leprosy patients, significantly so above endemic controls in lepromatous cases. In the sera of lepromatous leprosy (LL) patients who experienced an erythema nodosum leprosum (ENL) episode the SAP fell at the onset of ENL and remained low throughout, in two of three cases. Changes in SAP concentration parallel anti-sulphatide IgM concentrations. TH3, a monoclonal IgM germ-line antibody derived from a LL patient, and SAP share similar binding patterns. In this study we demonstrate binding to heparin and sulphatide. Moreover, SAP inhibited the binding of TH3 to sulphatide, as well as anti-sulphatide IgM found in a range of sera, and anti-sulphatide IgG in the only sera sample in which it was found. The observation that anti-TH3 idiotype monoclonal and polyclonal anti-SAP antibodies both inhibited the binding of TH3 and IgM in sera (but not IgG) to sulphatide without binding to sulphatide themselves further demonstrated similar binding specificities. The observations of similarity in binding reinforce ideas that SAP may function as a primitive opsonin, but the clear ability to inhibit binding of autoantibodies suggests that SAP may play a role in ameliorating tissue and particularly nerve damage in leprosy patients.     

芝麻花化学成分的研究

芝麻花为胡麻科植物胡麻Sesamum indicum L.(S.orientale L.)的干燥花,有生发、消肿之功效,可治疗脱发、冻疮、便秘等。民间单方芝麻花治疗寻常疣、扁平疣等,均有满意的疗效。目前人们对芝麻种子的化学成分研究较多,但尚没有人对芝麻花的化学成分进行系统研究。本研究运用硅胶柱、凝胶柱、反相柱等多种色谱方法进行分离纯化,并根据理化性质和波谱数据鉴定化合物的结构。从其乙醇提取物的乙酸乙酯和正丁醇部位分别得到10个化合物,分别鉴定为latifonin (1),苦瓜脑苷(momor-cerebroside,2),大豆脑苷II(soya-cerebroside II,3),1-O-β-D-葡糖-(2S,3S,4R,5E,9Z)-2-n-(2′-羟基二十四碳酰氨基)-1,3,4-三羟基-十八碳-5,9-二烯［1-O-β-D-glucopyranosyl-(2S,3S,4R,5E,9Z)-2-n-(2′-hydroxytetra-cosanoyl) 1,3,4-trihydroxy-5,9-octadienine,4］,1-O-β-D-葡糖-(2S,3S,4R,8Z)-2N-(2′-羟基二十四碳酰氨基)-3,4-二-羟基-8-十八碳烯［1-O-β-D-glucopyranosyl-(2S,3S,4R,8Z)-2-n-(2′R) 2′-hydroxytetracosanoyl)-3,4-dihydroxy-8-octadene,5］,2-乙酰橙酰胺［(2S,1″S)-aurantiamide acetate,6］,苯基乙醇-O-(2′-O-β-D-吡喃鼠李糖,3′-O-β-D-吡喃葡糖)-β-D-吡喃葡糖［benzyl alcohol-O-(2′-O-β-D-xylopyranosyl, 3′-O-β-D-glucopyranoside)-β-D-glucopyranoside,7］,β-谷甾醇(8),胡萝卜苷(9),D-半乳糖醇(10)。其中化合物4为新化合物,所有化合物均为首次从该植物的花中分离得到。     

海参和海星脑苷脂对大鼠急性肝损伤影响的比较研究

目的比较海参和海星脑苷脂对四氯化碳致大鼠急性肝损伤的影响。方法 24只大鼠按体重随机分为4组,分正常组、模型组、海参脑苷脂组(SCC)和海星脑苷脂组(SF),分别腹腔注射相应受试物10天,并于实验进行第7天开始利用四氯化碳(CCl4)建立急性肝损伤模型。测定了血清肝损伤指示酶活性和肝脏氧化应激水平以及肝脂含量,并观察了肝脏形态学变化,进行了DNA电泳分析。结果与正常组比,模型组大鼠血清中肝损伤指示酶活性均显著升高,肝脏脂质大量蓄积,肝脏中丙二醛(MDA)和一氧化氮(NO)含量显著增加,谷胱甘肽过氧化物酶(GSH-Px)和过氧化氢酶(CAT)活性显著降低,肝脏呈现明显病变,肝细胞大量坏死。海参脑苷脂组血清乳酸脱氢酶(LDH)、丙氨酸转氨酶(或称谷丙转氨酶ALT or GPT)、碱性磷酸酶(AKP)和天冬氨酸转氨酶(或称谷草转氨酶,AST or GOT)活性和肝脏MDA、NO含量以及肝脂含量相比模型组显著降低,GSH-Px和CAT活性显著升高,肝组织病变和肝细胞坏死状况明显改善,而海星脑苷脂组血清酶活和肝脏氧化应激水平变化不显著,肝脏脂肪含量显著降低。肝组织病变和肝细胞坏死改善效果相比海参脑苷脂组略差。结论海参脑苷脂对四氯化碳引发大鼠急性肝损伤的机制可能与其能够清除自由基,降低过氧化物,调节脂质代谢等作用有关,而海星脑苷脂对大鼠肝脏脂质蓄积表现出良好的改善效果,但其对肝损伤的作用机制仍有待深入研究。     

Sulfatide synthesis in mice with defective synthesis of 3'-phosphoadenosine 5'-phosphosulfate

The regulation of sulfation of galactocerebroside was examined in several tissues from brachymorphic mice, including the central nervous system and kidney. These animals have an inherited defect in the enzymes which synthesize 3'-phosphoadenosine 5'-phosphosulfate (PAPS) resulting in decreased sulfation of glycosaminoglycans. In contrast, sulfation of galactocerebroside to form sulfatide (sulfogalactocerebroside) was normal in brachymorphic mice even in tissues with decreased ability to synthesize PAPS. This suggests that, unlike the regulation of glycosaminoglycan sulfation, the formation of sulfatide is not stringently regulated by tissue levels of PAPS.