öλÔÓéÀֵǼÈë¿ÚÏÂÔØ

µÚ¶þÕ  Ö¬Àà  Lipids
Öص㣺Á×Ö¬¡¢ÌÇÖ¬
Ò»¡¢ Ö¬ÀàµÄ¸ÅÄî
²»ÈÜÓÚË®¶øÄܱ»ÒÒÃÑ¡¢Âȷ¡¢±½µÈ·Ç¼«ÐÔÓлúÈܼÁ³éÌá³öµÄ»¯ºÏÎͳ³ÆÖ¬Àà¡£Ö¬Àà°üÀ¨ÓÍÖ¬£¨¸ÊÓÍÈýÖ¬£©ºÍÀàÖ¬£¨Á×Ö¬¡¢À¯¡¢ÝÆÀà¡¢çÞÀࣩ¡£
¶þ¡¢ ·ÖÀà
£¨1£©µ¥´¿Ö¬£ºÖ¬·¾ËáÓë´¼ÀàÐγɵÄõ¥£¬¸ÊÓÍõ¥¡¢ÇÊÖ¬¡¢À¯
£¨2£©¸´ºÏÖ¬£º¸ÊÓÍÁ×Ö¬¡¢ÇÊÁ×Ö¬¡£
£¨3£©ÝÆÀàºÍçÞÀ༰ÆäÑÜÉúÎ²»º¬Ö¬·¾Ëᣬ¶¼ÊÇÒìÎì¶þÏ©µÄÑÜÉúÎï¡£
£¨4£©ÑÜÉúÖ¬£ºÉÏÊöÖ¬ÀàµÄË®½â²úÎ°üÀ¨Ö¬·¾Ëá¼°ÆäÑÜÉúÎï¡¢¸ÊÓÍ¡¢ÇÊ°±´¼µÈ¡£
£¨5£©½áºÏÖ¬ÀࣺÌÇÖ¬¡¢Ö¬µ°°×
Èý¡¢ Ö¬ÀàµÄÉúÎïѧ¹¦ÄÜ
Ö¬ÀàµÄÉúÎïѧ¹¦ÄÜÒ²¶àÖÖ¶àÑù£º
¢ÙÉúÎïĤµÄ½á¹¹×é·Ö(¸ÊÓÍÁ×Ö¬ºÍÇÊÁ×Ö¬£¬µ¨¹Ì´¼¡¢ÌÇÖ¬)£»¢ÚÄÜÁ¿Öü´æÐÎʽ(¶¯Îï¡¢ÓÍÁÏÖÖ×ӵĸÊÓÍÈýõ¥)£»¢Û¼¤ËØ¡¢Î¬ÉúËغÍÉ«ËصÄÇ°Ìå(ÝÆÀà¡¢¹Ì´¼Àà)£»¢ÜÉú³¤Òò×Ó£»¢Ý¿¹Ñõ»¯¼Á£»¢Þ »¯Ñ§ÐźÅ(Èç   )£»¢ß²ÎÓëÐźÅʶ±ðºÍÃâÒߣ¨ÌÇÖ¬£©£»¢à¶¯ÎïµÄÖ¬·¾×éÖ¯Óб£Î£¬·À»úеѹÁ¦µÈ±£»¤¹¦ÄÜ£¬Ö²ÎïµÄÀ¯ÖÊ¿ÉÒÔ·Àֹˮ·ÖµÄÕô·¢¡£
µÚÒ»½Ú Ö¬·¾Ëá¼°ÆäÑÜÉúÎï
Ò»¡¢ Ö¬·¾Ëá
¾ø´ó¶àÊýµÄÖ¬·¾ËẬÓÐżÊý¸ö̼ԭ×Ó£¬Ðγɳ¤¶ø²»·ÖÖ§µÄÁ´(Ò²ÓзÖÖ§µÄ»òº¬»·µÄÖ¬·¾Ëá)¡£
²»±¥ºÍÖ¬·¾ËáÓÐ˳ʽºÍ·´Ê½Á½ÖÖÒìÎïÌå¡£µ«ÉúÎïÌåÄÚ´ó¶àÊýÊÇ˳ʽ½á¹¹¡£
²»±¥ºÍÖ¬·¾ËáÖУ¬·´Ê½Ë«¼ü»áÔì³ÉÖ¬·¾ËáÁ´ÍäÇú£¬·Ö×Ó¼äûÓб¥ºÍÖ¬·¾ËáÁ´ÄÇÑù½áºÏ½ôÃÜ¡£Òò´Ë£¬²»±¥ºÍÖ¬·¾ËáµÄÈÛµãµÍ¡£
Ö¬·¾Ëá(Ö÷ÒªÊǶ¹Þ¢ËáÓë×ØéµËá)¿ÉÒÔÓëµ°°×Öʹ²¼ÛÏàÁ¬£¬ÐγÉÖ¬õ£µ°°×(acyloted protein)£¬Ö¬õ£»ùÍÅÄÜ´Ù½øĤµ°°×ÓëÊèË®»·¾³¼äµÄÏ໥×÷Óá£
1¡¢±ØÐèÖ¬·¾Ëá  essential  fatty  acids
Ö²ÎïºÍϸ¾ú¿ÉÒÔÀûÓÃÒÒõ£CoAºÏ³ÉËùÐèµÄÈ«²¿Ö¬·¾Ëá¡£
²¸È鶯Îï¼È¿ÉÒÔ´ÓʳÎïÖлñµÃ´ó²¿·ÖÖ¬·¾ËᣬҲ¿ÉÒԺϳɱ¥ºÍÖ¬·¾ËáºÍһЩµ¥²»±¥ºÍÖ¬·¾Ëá¡£
µ«ÊÇ£¬²¸È鶯Îï²»Äܺϳɶ಻±¥ºÍÖ¬·¾ËᣨÈçÑÇÓÍËáºÍÑÇÂéËᣩ£¬³ÆΪ±ØÐèÖ¬·¾Ëá¡£
ÑÇÓÍËáºÍÑÇÂéËá±ØÐë´ÓÖ²ÎïÖлñÈ¡¡£»¨ÉúËÄÏ©Ëá¿ÉÓÉÑÇÓÍËáÔÚÌåÄںϳÉ¡£

P52 ±í2¡ª3ijЩÓÍÖ¬µÄÖ¬·¾Ëá×é³É

2¡¢Ôí»¯Öµ£¨ÆÀ¹ÀÓ͵ÄÖÊÁ¿£©
ÍêÈ«Ôí»¯1¿ËÓÍÖ¬ËùÐèKOHµÄºÁ¿ËÊý£¬³ÆÔí»¯Öµ¡£
ÓÃÀ´ÆÀ¹ÀÓÍÖ¬µÄÖÊÁ¿¡£
3¡¢ËáÖµ£¨Ëá°Ü³Ì¶È£©
ÖкÍ1¿ËÓÍÖ¬ÖеÄÓÎÀëÖ¬·¾ËáËùÏûºÄµÄKOHºÁ¿ËÊý¡£
4¡¢£¨²»±¥ºÍ¼üµÄ¶àÉÙ£©
100¿ËÓÍÖ¬ÎüÊÕµâµÄ¿ËÊý¡£
¶þ¡¢ Àà¶þÊ®ÍéËá
Ò²³ÆÀ໨ÉúËᣨeicosanoid£©£¬°üÀ¨Ç°ÁÐÏÙËØÀà(prostaglandin)£¬ÄýѪ¶ñÍéÀà(thromboxane)ºÍ°×ϸ°ûÈýÏ©Àà(leucotriene)
ÊÇÒ»´óÀàÓÉÐí¶à²¸È鶯Îï×éÖ¯²úÉúµÄ¼¤ËØÀàµÄÎïÖÊ¡£ËüÃÇÖ»ÔÚ²úÉúµÄÆ÷¹ÙÖÐÆð×÷Óã¬ËùÒÔ³ÆΪ×ÔÃÚµ÷¿Ø·Ö×Ó£¬¶ø²»ÊǼ¤ËØ¡£
´ó¶àÊýµÄÀà¶þÊ®ÍéËáÊÇ»¨ÉúËÄÏ©ËáµÄÑÜÉúÎï¡£
»¨ÉúËÄÏ©ËáÒ²³Æ5,8,11,14-¶þʮ̼ËÄÏ©Ëá(eicosatetraenoio acid)£¬ÊÇÓÉÑÇÓÍËáºÏ³Éºó¼ÓÉÏÒ»¸ö¶þ̼µ¥Î»¡¢ÒýÈëÁ½¸öË«¼ü¡£
1¡¢ Ç°ÁÐÏÙËØÀà
ͼ9A
Ç°ÁÐÏÙËØÀàÊÇ»¨ÉúËÄÏ©µÄÑÜÉúÎï¡£
Ç°ÁÐÏÙËØÀàÓÐÒ»¸ö»·ÎìÍé½á¹¹£¬C11¡¢C15λµã¸÷ÓÐÒ»¸ö-OH¡£
PGEÔÚC9λÉÏÓÐÒ»¸öC=O(carbonyl group)£¬PGFÔÚC9ÉÏÓÐÒ»¸ö-OH¡£
½Ç×¢Êýѧ±íÃ÷·Ö×ÓÖÐË«¼üµÄÊýÄ¿£¬PG2ÀàÇ°ÁÐÏÙËØÊÇÈËÀàÖÐ×îÖØÒªµÄÇ°ÁÐÏÙËØ¡£
Ç°ÁÐÏÙËزÎÓëÐí¶àÉúÀí¹ý³ÌµÄµ÷½Ú¿ØÖÆ£¬´Ù½øÑ×Ö¢·´Ó¦£¬²ÎÓëÉúÖ³¹ý³Ì(ÈçÅÅÂÑ¡¢ÊÜÔкͷÖÃäʱ×Ó¹¬µÄÊÕËõ)£¬²ÎÓëÏû»¯¡£

ͼ9B
2¡¢ ¡¢ÄýѪ¶ñÍéÀà(thromboxanes)
ÄýѪ¶ñÍéÀàÒ²ÊÇ»¨ÉúËÄÏ©ËáµÄÑÜÉúÎï¡£
ÓëÆäËûÀà¶þÊ®ÍéË᲻ͬµÄÊÇÄýѪ¶ñÍéÀàÓл·ÃѵĽṹ¡£
ÄýѪ¶ñÍéA2(TxA2)ÊǸÃÀ໯ºÏÎïÖÐ×îÖØÒªµÄÒ»ÖÖ£¬ËüÖ÷ÒªÓÉѪС°å²úÉú£¬´Ù½øѪС°åÄý¾ÛºÍƽ»¬¼¡ÊÕËõ¡£
3¡¢ °×ϸ°ûÈýÏ©£¨leucotriene,LT£©
ÊÇ»¨ÉúËÄÏ©ËáµÄôÇ»ùÖ¬·¾ËáÑÜÉúÎï¡£
×î³õÊÇÔÚ°×ϸ°ûÖз¢Ïֵģ¬²¢ÇÒÓÐÈýÏ©½á¹¹£¬¹ÊÃû°×ϸ°ûÈýÏ©¡£
LTC4¡¢LTD4ºÍLTE4ÊǹýÃôÐÔ·´Ó¦µÄÂý·´Ó¦ÎïÖʵÄ×é·Ö£¬ÔÚÑ×Ö¢·´Ó¦Æð»ý¼«×÷Ó㬴ٽø°×ϸ°ûÇ÷ÏòÆÆ»µ×éÖ¯¡£
µÚ¶þ½Ú Ö¬õ£¸ÊÓÍ
ÒòΪ²»´øµçºÉ£¬ÓÐʱҲ³ÆÖÐÐÔÖ¬(neutral fats)
½á¹¹£º
ͼ1
¼òµ¥ÈýÖ¬õ£¸ÊÓÍ
»ìºÏÈýÖ¬õ£¸ÊÓÍ
µÚÈý½Ú Á×Ö¬
Á×Ö¬ÊÇÖØÒªµÄÁ½Ç×ÎïÖÊ£¬ËüÃÇÊÇÉúÎïĤµÄÖØÒª×é·Ö¡¢È黯¼ÁºÍ±íÃæ»îÐÔ¼Á(±íÃæ»îÐÔ¼ÁÊÇÄܽµµÍÒºÌ壬ͨ³£ÊÇË®µÄ£¬±íÃæÕÅÁ¦£¬ÑØË®±íÃæÀ©É¢µÄÎïÖÊ)¡£
Á×Ö¬ÓÐÁ½Àࣺ¸ÊÓÍÁ×Ö¬ºÍÇÊ°±´¼Á×Ö¬¡£
¸ÊÓÍÁ×Ö¬ÓɸÊÓÍ¡¢Ö¬·¾Ëá¡¢Á×ËáºÍÒ»·Ö×Ó°±»ù´¼(È絨¼î¡¢ÒÒ´¼°·¡¢Ë¿°±Ëá»ò¼¡´¼)×é³É¡£
ÇÊ°±´¼Á×Ö¬Ö»ÊÇÒÔÇÊ°±´¼´úÌæÁ˸ÊÓÍ¡£
Ò»¡¢ ¸ÊÓÍÁ×Ö¬
ÌìÈ»´æÔڵĸÊÓÍÁ×Ö¬¶¼ÊÇL¡ª¹¹ÐÍ¡£
1¡¢ ½á¹¹Óë·ÖÀà
ÒÀÕÕ°±»ù´¼µÄ²»Í¬¿É·ÖÒÔϼ¸Àࣺ

P57 ±í2-6¸÷ÖÖ¸ÊÓÍÁ×Ö¬µÄ¼«ÐÔÍ·²¿ºÍµçºÉÁ¿
£¨1£©¡¢ Á×Ö¬õ£µ¨¼î£¨ÂÑÁ×Ö¬£©£¨PC£©
HO¡ªCH2CH2N+£¨CH3£©3£¨µ¨¼î£©

·Ö²¼£º
Ö²Î´ó¶¹µÈ£¬
¶¯ÎÄÔ¡¢¾«Òº¡¢ÉöÉÏÏÙ¡¢ºìϸ°û£¬µ°Âѻƣ¨8-10%£©¡£
×÷Ó㺿ØÖƸÎÖ¬´úл£¬·ÀÖ¹Ö¬·¾¸ÎµÄÐγÉ¡£
£¨2£©¡¢ Á×Ö¬õ£ÒÒ´¼°·£¨ÄÔÁ×Ö¬£©£¨PE£©
HO¡ªCH2CH2¡ªN+H3£¨ÒÒ´¼°·£©
²ÎÓëѪҺÄý½á¡£
£¨3£©¡¢ Á×Ö¬õ£Ë¿°±ËᣨPS£©
HO¡ªCH2CH¡ªCOO-£¨Ë¿°±Ëᣩ
              N+H3
£¨1£©¡ª£¨3£©X¾ùΪ°±»ù´¼¡£
£¨4£©¡¢ Á×Ö¬õ£¼¡´¼£¨PI£©
        Í¼

£¨5£©¡¢ Á×Ö¬õ£¸ÊÓÍ£¨PG£©
£¨6£©¡¢ ¶þÁ×Ö¬õ£¸ÊÓÍ£¨ÐÄÁ×Ö¬£©
2¡¢ ¸ÊÓÍÁ×Ö¬µÄÐÔÖÊ
¢Ù¼«ÐÔ£º¼«ÐÔÍ·¡¢·Ç¼«ÐÔβ
¢Ú´øµçÐÔ£¨¿ÉÓÃÓÚ·ÖÀë´¿»¯£©
        Í¼  
¶þ¡¢ ÇÊÁ×Ö¬
¸ßµÈ¶¯Îï×éÖ¯Öк¬Á¿½Ï·á¸»¡£
1¡¢ ×é³É£º
Ò»¸öÇÊ°±´¼          Ò»¸öÖ¬·¾Ëá          Ò»¸öÁ×Ëá          Ò»¸öµ¨¼î»òÒÒ´¼°·
2¡¢ ½á¹¹ÓëÐÔÖÊ
ÇÊÁ×Ö¬¼«ÐÔÍ·²¿·ÖÊÇÁ×Ö¬õ£µ¨¼î»òÁ×Ö¬õ£ÒÒ´¼°·¡£
ÇÊÁ×Ö¬½á¹¹Óë¸ÊÓÍÁ×Ö¬ÏàËÆ£¬Òò´ËÐÔÖÊÓë¸ÊÓÍÁ×Ö¬»ù±¾Ïàͬ¡£
µÚËÄ½Ú ÇÊÖ¬Àà
ÇÊÖ¬ÀàÒ²ÊǶ¯Ö²ÎïÉúÎïĤµÄÖØÒª×é·Ö¡£
ÇÊÖ¬ÀຬÓÐÒ»¸ö³¤µÄ°±»ù´¼¡£
Ò»¡¢ ÇÊ°±´¼
ÒÑ·¢ÏÖµÄÇÊ°±´¼ÀàÔ¼ÓÐ30ÖÖ¡£

ͼ  2-°±»ù-4-Ê®°Ë̼ϩ-1.3-¶þ´¼
´ËË«¼ü»¹Ô­£¬¼´¶þÇâÇÊ°±´¼


ÇÊ°±´¼              Ö²ÎïÇÊ°±´¼          
¶þ¡¢ Éñ¾­õ£°·
ÇÊÖ¬ÀàµÄºËÐĽṹÊÇÉñ¾­õ£°·£¨ceramide£©£¬ÓÉÇÊ°±´¼°±»ùÒÔõ£°·¼üÓ볤Á´£¨18¡ª26C£©Ö¬·¾ËáµÄôÇ»ùÏàÁ¬¡£

  


Éñ¾­õ£°·
ÔÚÇÊÁ×Ö¬ÖУ¬Éñ¾­õ£°·1λµÄ-OH±»Á×Ëᵨ¼î(phosphorylcholine)»òÁ×ËáÒÒ´¼°·(phosphorylethanolamine)µÄÁ×Ëá»ùÒòõ¥»¯¡£
³ýÁ˶¯Îïϸ°ûĤÍ⣬ÇÊÁ×Ö¬ÔÚÉñ¾­Ï¸°ûµÄËèÇÊÖк¬Á¿×î·á¸»¡£
µÚÎå½Ú ½áºÏÖ¬Àà
Ò»¡¢ ÌÇÖ¬ glycolipid  
P478 ͼ9¡ª8   P479 ͼ9¡ª10

¸ÊÓÍ´¼ÌÇÖ¬        N¡ªÖ¬õ£Éñ¾­ÇÊ°±´¼ÌÇÖ¬£¨Éñ¾­õ£°·ÌÇÖ¬£©
1¡¢ ¸ÊÓÍ´¼ÌÇÖ¬
        Í¼
°ëÈéÌǸÊÓͶþõ¥      ³Æ£º6¡ª»Ç»ùGlc¸ÊÓͶþõ¥
2¡¢ N¡ªÖ¬õ£Éñ¾­ÇÊ°±´¼ÌÇÖ¬£¨Éñ¾­õ£°·ÌÇÖ¬£©
Éñ¾­õ£°·»¹ÊÇÌÇÖ¬µÄÇ°ÌåÎÓÐʱ³ÆÇÊÌÇÖ¬¡£


ͼ9.9
ÔÚÇÊÌÇÖ¬ÖУ¬µ¥ÌÇ¡¢Ë«ÌÇ»ò¹ÑÌÇͨ¹ýO-ÌÇÜÕ¼üÓëÉñ¾­õ£°·ÏàÁ¬£¬ÖØÒªµÄÇÊÌÇÖ¬ÓÐÄÔÜÕÖ¬(cerebroside)¡¢ÁòÄÔÜÕÖ¬(sulfatide)ºÍÉñ¾­½ÚÜÕÖ¬(ganglioside)¡£
ÄÔÜÕÖ¬Êǵ¥ÌÇÓëÉñ¾­õ£°·ÐγɵÄÌÇÖ¬£¬ÊÇ·ÇÀë×ÓÐ͵Ä¡£°ëÈéÌÇÄÔÜÕÖ¬(galatocerebroside)¼¸ºõÈ«²¿´æÔÚÓÚÄÔµÄϸ°ûĤÖС£
ÄÔÜÕÖ¬±»ÁòËữºó³ÆΪÁòÄÔÜÕÖ¬£¬ÔÚÉúÀípHÏ´ø¸ºµçºÉ¡£
¹ÑÌÇÁ´(´øÓÐÒ»¸ö»ò¶à¸öÍÙÒºËá²Ð»ù)ÓëÉñ¾­õ£°·ÐγɵÄÇÊÌÇÖ¬³ÆΪÉñ¾­½ÚÜÕÖ¬£¬×î³õÊÇ´ÓÉñ¾­×éÖ¯ÖзÖÀëµ½µÄ£¬ÔÚÆäËü×éÖ¯ÖÐÒ²Óзֲ¼¡£
Éñ¾­½ÚÜÕÖ¬µÄÃüÃûº¬ÓÐM¡¢D¡¢TºÍ½Ç×¢Êý×Ö£¬M¡¢D¡¢T·Ö±ð±íʾº¬ÓÐÒ»¸ö¡¢Á½¸ö¡¢Èý¸öÍÙÒºËᣬÊý×Ö±íʾÔÚÌÇÁ´ÉϵÄλÖá£
£¨1£©¡¢ ÄÔÜÕÖ¬£¨ÖÐÐÔÌÇÇÊÖ¬Àࣩ

ͼ
Ö÷ÒªÔÚÉñ¾­¡¢ÄÔ×éÖ¯ÖУ¬XΪGlc³ÆGlcÄÔÜÕÖ¬£¬XΪGal³ÆGalÄÔÜÕÖ¬¡£X»¹¿ÉÄÜÊÇ£ºFuc¡¢GlcNAc¡¢GalNAc
£¨2£©¡¢ Éñ¾­½ÚÜÕõ¥£¨ËáÐÔÌÇÇÊÖ¬Àࣩ
º¬ÓÐÍÙÒºËᣬÔÚÄÔ»ÒÖʺÍÐØÏÙÖк¬Á¿¸ß¡£
ÖÐÊàÉñ¾­ÏµÍ³Ä³Ð©Éñ¾­ÔªÄ¤µÄÌØÕ÷ÐÔÖ¬£¬¿ÉÄÜÓëͨ¹ýÉñ¾­ÔªµÄÉñ¾­³å¶¯´«µÝÓйØ¡£

ͼ
È˵ÄÉñ¾­ÏµÍ³Ï¸°ûĤÖÁÉÙÓÐ15ÖÖÉñ¾­½ÚÜÕÖ¬£¬ËüÃǵÄÉúÎ﹦ÄÜÉÐδÍêÈ«Á˽â¡£
3¡¢ ÌÇÖ¬µÄÉúÎïѧ¹¦ÄÜ
ÌÇÖ¬µÄ¹¦ÄÜ»¹²»Ê®·ÖÇå³þ£¬ÓÐЩ¶¯Îïϸ°ûĤÉϵÄÌÇÖ¬·Ö×ÓÄÜÓëϸ¾ú¶¾ËØÒÔ¼°Ï¸¾úϸ°û½áºÏ£¬ÆðÊÜÌåµÄ×÷Óá£
£¨1£©Ï¸°û½á¹¹µÄ¸ÕÐÔ
£¨2£©¿¹Ô­µÄ»¯Ñ§±ê¼Ç  ÑªÐÍ¿¹Ô­
        Í¼
È˵ÄA¡¢B¡¢OѪÐͲîÒìÔÚÓÚÌÇÁ´Ä©¶Ë²Ð»ù¡£ÏÖÔÚÁÙ´²ÉÏÕýÑо¿ÓÃø´Ù½µ½âB¡ª¿¹Ô­»òA¿¹Ô­µÄÄ©¶Ë²Ð»ùGal»òGalNAc£¬´Ó¶øÔö¼ÓO¡ª¿¹Ô­µÄѪҺÀ´Ô´¡£
£¨3£©Ï¸°û·Ö»¯½×¶Î¿É¼ø¶¨µÄ»¯Ñ§±ê¼Ç
¿ÉÄÜÓëÌÇÁ´µÄ³¤¶ÌÓйØ
£¨4£©µ÷½Úϸ°ûµÄÕý³£Éú³¤
ÓëÕý³£Ï¸°ûת»¯³ÉÖ×°©Ï¸°ûÓйØ¡£Ö×°©CellµÄÉñ¾­½ÚÜÕÖ¬ÌÇÁ´±ÈÕý³£CellµÄ¶Ì¡£
£¨5£©ÊÚÓèϸ°ûÓëÆäËüÉúÎï»îÐÔÎïÖʵķ´Ó¦ÐÔÇãÏò¡£

u ÇÊÖ¬Öü»ý²¡(sphingolipld storage disease, sphingolipidose)
ÈÜøÌåÖü»ý²¡ÊÇÓÉÓÚ½µ½âijÖÖÌض¨´úлÎïµÄø·¢ÉúÒÅ´«ÐÔȱÏÝÔì³ÉµÄ¡£Ò»Ð©ÈܽâÌåÖü»ý²¡ÓëÇÊÖ¬´úлÓйØ£¬Ò²³ÆÇÊÖ¬Öü»ý²¡£¬³£¼ûµÄ¾ÍÊÇTay-sochsÉñ¾­½ÚÜÕGM2Öü»ý²¡£¬ÕâÊÇÓÉÓÚ½µ½âËüµÄb-hexosaminidaseA(b-°±»ù¼ºÌÇÜÕø)ȱÏÝÔì³ÉµÄ¡£µ±Ï¸°û»ýÀÛGM2ʱ¾ÍÈÜÕÍ×îÖÕËÀÍö£¬Tay-Sachs×ÛºÏÖ¢(ʧÃ÷£¬¼¡ÈâήËõ£¬³é´¤£¬¾«Éñ´íÂÒ)£¬Í¨³£ÔÚ³öÉúÊýÔºó±íÏÖ³öÀ´¡£
disease symptern Accumulating spluingolipid Enzyme deficiency
Tay-sachs disease BlindnessMuscle weaknessSeizuresMental retardation Ganglioside GM2 b-hexosaminidoseA
Gaucheris disease Mental retardations,Liver and spleen enlargementEresion of cong bones Glucocerebosile b-glucosidase
Niemann-Pick disease Montal retardution sphingomyelin sphingomylinase
¶þ¡¢ Ö¬µ°°×   lipoprotein
Òªµã£ºÑª½°Ö¬µ°°× Ѫ½°°×µ°°×
£¨Ñ§Éú×Ô¼º¿´£¬´Ë´¦²»½²£¬ÔÚÖ¬´úлÖн²¡££©

ËäȻ֬µ°°×¿ÉÒÔÖ¸ÈκÎÓëÖ¬»ù(ÈçÖ¬·¾Ëá¡¢ÒìÎì¶þÏ©)¹²¼ÛÏàÁ¬µÄµ°°×¡¢µ«Ëü³£³£ÓÃÀ´Ö¸²¸È鶯ÎïѪ½¬(ÓÈÆäÊÇÈË)ÖеÄÖ¬-µ°°×Öʸ´ºÏÎï¡£
Ѫ½¬Ö¬µ°°×¿ÉÒÔ°ÑÖ¬Àà(Èýõ£¸ÊÓÍ¡¢Á×Ö¬¡¢µ¨¹Ì´¼)´ÓÒ»¸öÆ÷¹ÙÔËÊäµ½ÁíÒ»¸öÆ÷¹Ù¡£


ͼ9.17  P233
Ѫ½¬Ö¬µ°°×¸ù¾ÝÃܶÈÀ´·ÖÀࣺ
£¨1£© ÈéÃÓ΢Á££¬Ãܶȷdz£µÍ£¬ÔËÊä¸ÊÓÍÈýõ¥ºÍµ¨¹Ì´¼Ö¬£¬´ÓС³¦µ½×éÖ¯¼¡ÈâºÍadipose×éÖ¯¡£
£¨2£© ¼«µÍÃܶÈÖ¬µ°°×VLDL(0.95-1.006g/cm3)£¬ÔÚ¸ÎÔàÖÐÉú³É£¬½«Ö¬ÀàÔËÊäµ½×éÖ¯ÖУ¬µ±VLDL±»ÔËÊ䵽ȫÉí×é֯ʱ£¬±»·Ö½âΪÈýõ£¸ÊÓÍ¡¢ÍѸ¨»ùµ°°×ºÍÁ×Ö¬£¬×îºó£¬VLDL±»×ª±äΪµÍÃܶÈÖ¬µ°°×¡£
£¨3£© µÍÃܶÈÖ¬µ°°×(LDL£¬1.006-1.063g/cm3)£¬°Ñµ¨¹Ì´¼ÔËÊäµ½×éÖ¯£¬¾­¹ýһϵÁи´ÔӵĹý³Ì£¬LDLÓëLDLÊÜÌå½áºÏ²¢±»Ï¸°ûÍÌʳ¡£
£¨4£© ¸ßÃܶÈÖ¬µ°°×(HDL£¬1.063-1.210g/cm3)£¬Ò²ÊÇÔÚ¸ÎÔàÖÐÉú³É£¬¿ÉÄܸºÔðÇå³ýϸ°ûĤÉϹýÁ¿µÄµ¨¹Ì´¼¡£µ±Ñª½¬ÖеÄÂÑÁ×Ö¬£ºµ¨¹Ì´¼õ£»ùתÒÆø(Lecithin cholesterol acyltransferase, LCAT)½«ÂÑÁ×Ö¬ÉϵÄÖ¬·¾Ëá²Ð»ùתÒƵ½µ¨¹Ì´¼ÉÏÉú³Éµ¨¹Ì´¼Ö¬Ê±£¬HDL½«ÕâЩµ¨¹Ì´¼Ö¬¶¯Êäµ½¸Î¡£¸ÎÔཫ¹ýÁ¿µÄµ¨¹Ì´¼×ª»¯Îªµ¨Ö­Ëá¡£

u Ö¬µ°°×Ó붯ÂöÖàÑùÓ²»¯£º(atherosclerosis)
¶¯ÂöÖàÑùÓ²»¯ÊÇÒ»¸öÂýÐÔ²¡£¬Ôڴ˹ý³ÌÖУ¬ÖàÑùÎïÖÊÖð½¥³Á»ýÔÚ¶¯ÂöµÄÄÚ±ÚÉÏ£¬ÕâЩ³Á»ýÎï³ÆΪPlaque(Ê´°ß)£¬ÔÚplaqueÐγɹý³ÌÖУ¬Æ½»¬¼¡Ï¸°û¡¢¾ÞÊÉϸ°ûºÍ¸÷ÖÖϸ°û²ÐÔüÖð½¥¾Û¼¯¡£µ±¾ÞÊÉϸ°ûÖÐÍÌʳÁË´óÁ¿Ö¬ÀàÎïÖÊ(Ö÷ÒªÊǵ¨¹Ì´¼ºÍµ¨¹Ì´¼Ö¬)ËüÃǾͳÉΪÖàÑù»¯Ï¸°û¡£×îºó£¬ÖàÑùÓ²»¯°ß¸Æ»¯(calcify)Í»È붯ÂöÇ»£¬×èֹѪҺÁ÷¶¯£¬´óÄÔ¡¢ÐÄ¡¢·ÎµÈÆ÷¹Ù¾Í»áȱÑõºÍÓªÑø¡£¹Ú×´¶¯ÂöÖàÑùÓ²»¯²¡ÊÇ×î³£¼ûµÄÒ»ÖÖ£¬ÓÉÓÚȱÑõºÍÓªÆÆ»µÁËÐļ¡¡£
PlaqueÖеĵ¨¹Ì´¼´ó²¿·ÖÊÇÀ´×ÔÖàÑùϸ°ûÍÌÊɵÄLDL¡£Òò´Ë£¬ºÁ²»Ææ¹Ö£¬¸ßˮƽµÄѪ½¬LDLÓë¹Ú×´¶¯ÂöÖàÑùÖ¢Ö±½ÓÏà¹Ø(LDLº¬ÓдóÁ¿µÄµ¨¹Ì´¼¼°µ¨¹Ì´¼Ö¬)£¬ÆäËüÏà¹ØÒòËØ»¹°üÀ¨¸ßÖ¬ÀàÒûʳ¡¢ÎüÑÌ¡¢ÒÖÓôºÍȱÉÙÔ˶¯£¬¸ßˮƽµÄѪ½¬HDLÓë´ú¼¸ÂʵĹÚ×´¶¯Âö²¡ÓйØ¡£¸Îϸ°ûÊÇΨһ¾ßÓÐHDLÊÜÌåµÄϸ°û¡£
¿ÉÖàÑù»¯µÄϸ°û¾ßÓÐLDLÊÜÌ壬µ±LDLÓëÊÜÌå½áºÏºóÕâЩϸ°û¾Íͨ¹ý°ûÍÌ×÷ÓÃÍÌʳLDL¡£ÔÚÕý³£Çé¿öÏ£¬½øÈëϸ°ûÖеÄLDLÊͷųöµÄµ¨¹Ì´¼ºÍÆäËüÖ¬Àà¿ÉÓÃÓÚϸ°û½á¹¹ºÍ´úлÉϵÄÐèÒª¡£Í¨³£Çé¿öÏÂLDLÊÜÌ幦ÄÜÊǸ߶ȵ÷¿ØµÄ£¬ÍÌÈëÏà¶Ô´óÁ¿µÄLDLºó£¬LDLÊÜÌåºÏ³É¾Í½µµÍ¡£¾ÞÊÉϸ°ûÈ´²»Í¬£¬LDLÊÜÌåµÄºÏ³É²¢²»½µµÍ£¬ÖàÑùÓ²»¯°ßÖеľÞÊÉϸ°ûº¬ÓиßˮƽµÄLDLÊÜÌ壬¶øÇÒ¶ÔÑõ»¯ÆÆ»µµÄLDLÈÔÓÐÇ׺ÍÁ¦¡£¿¹»µÑªËá(Vc)ºÍVE¶¼ÊÇ¿¹Ñõ»¯¼Á£¬ÄÜÒÖÖ¹ÖàÑù°ßµÄÐγÉ¡£


µÚÁù½Ú ÝÆÀàºÍ¹Ì´¼À໯ºÏÎï
¿ÉÒÔͳ³ÆΪÀàÒìÎì¶þÏ©Àà(isoprenoid)£¬ÓÉÒÒõ£-CoA¾­ÓÉÒìÎì¶þÏ©½¹Á×ËáÉú³ÉµÄ£¬¶ø²»ÊÇÓÉÒìÎì¶þÏ©ºÏ³ÉµÄ
Ò»¡¢ ÝÆÀà
һЩÕæºËµ°°×Öʺϳɺ󾭹ýÒìÎì¶þÏ©»¯£¬³£¼ûµÄÒìÎì¶þÏ©»ùÍžÍÊÇfarnesylºÍgeranylgeranyl group
¶þ¡¢ ¹Ì´¼Àà
½á¹¹£º
º¬Óл·ÎìÍé¶àÇâ·ÆĸºËµÄÒ»Àà´¼¡¢Ëá¼°ÆäÑÜÉúÎï¡£°üÀ¨£º¹Ì´¼¡¢¹Ì´¼ÑÜÉúÎï¡£

ͼ
1¡¢ µ¨¹Ì´¼£¨¶þÇ⵨¹Ì´¼¡¢T¡ªÍÑÇ⵨Ëá¡¢µ¨¹Ì´¼õ¥£©
ÒÔÓÎÀë»òõ¥µÄÐÎ̬´æÔÚÓÚÒ»Çж¯Îï×éÖ¯ÖУ¬Ö²ÎïÖÐûÓС£ÊÇ×îÔçÓÉ´Ó¶¯ÎﵨʯÖзÖÀë³öµÄ¹Ì´¼¡£
£¨1£©¡¢ ½á¹¹
C3 ôÇ»ù
C10ºÍC13¸÷Ò»¸ö¼×»ù
C5ÓëC6¼äÒ»¸öË«¼ü
C17ÒìÐÁÍé
£¨2£©¡¢ ÐÔÖÊ
°×É«¡¢Ð±·½¾§Ìå¡£
a. ´¼»ù¿ÉÓëÖ¬Ëá³Éõ¥£¨×ØéµËá¡¢Ó²Ö¬Ëá¡¢ÓÍËᣩ
b. Ë«¼ü¿É¼ÓÇâ
£¨3£©¡¢ ·Ö²¼¼°¹¦ÄÜ
a. 70ǧ¿ËÈËÌ庬140¿Ë×óÓÒ£¬1/4ÔÚÄÔ¼°Éñ¾­×éÖ¯ÖУ¬¸Î¡¢Éöº¬Á¿½Ï¶à¡£ÉöÉÏÏÙ¡¢Âѳ²µÈºÏ³É¹Ì´¼¼¤ËصÄÏÙÌ庬Á¿Ò²½Ï¶à£¬¿É´ï1¡ª5%¡£ÑªÇåÖк¬Á¿Éý¸ß£¬»áÔö¼Ó»¼ÐÄѪ¹Ü¼²²¡µÄ¿ÉÄÜÐÔ¡£
b. µ¨¹Ì´¼ÊÇÉúÎïĤµÄÖØÒª³É·Ö£¬ôÇ»ù¼«ÐԶ˷ֲ¼ÓÚĤµÄÇ×Ë®½çÃ棬ĸºË¼°²àÁ´ÉîÈëĤ˫²ã£¬¿ØÖÆĤµÄÁ÷¶¯ÐÔ£¬×èÖ¹Á×Ö¬ÔÚÏà±äζÈÒÔÏÂʱת±ä³É½á¾§×´Ì¬£¬±£Ö¤Ä¤ÔÚµÍÎÂʱµÄÁ÷¶¯ÐÔ¼°Õý³£¹¦ÄÜ¡£
c. µ¨¹Ì´¼ÊǺϳɵ¨Ö­Ëá¡¢Àà¹Ì´¼¼¤ËØ¡¢Î¬ÉúËØDµÈÉúÀí»îÐÔÎïÖʵÄÇ°Ìå¡£
µ¨Ö­ËᣨÔÚ¸ÎÖкϳɣ©²ÎÓ볦µÀÖ¬ÀàÎüÊÕ
ÉöÉÏÏÙƤÖʼ¤ËØ¡¢´Æ¼¤ËØ¡¢ÐÛ¼¤ËØ
               7Ò»ÍÑÇ⵨¹Ì´¼   ×ÏÍâÏß   άÉúËØD3
2¡¢ Ö²Îï¹Ì´¼
²»Äܱ»¶¯ÎïÎüÊÕºÍÀûÓá£
Ö÷ÒªÓУº¶¹¹Ì´¼£¨´ó¶¹ÖУ©
Âó¹Ì´¼£¨ÂóÑ¿ÖУ©
3¡¢ ½Íĸ¹Ì´¼
Âó½Ç¹Ì´¼£¬¾­×ÏÍâ¹âÕÕÉä¿Éת»¯³ÉάÉúËØD3¡£
Èý¡¢ ¹Ì´¼ÑÜÉúÎï
1¡¢ µ¨Ö­Ëá
ͼ
¶àÊý¼¹×µ¶¯ÎïµÄµ¨ËᣬÄÜÒÔëļüÓëGly»òÅ£»ÇËá½áºÏ¡£µ¨ËáÓëÖ¬·¾Ëá»òÆäËûÖ¬Àà½áºÏ£¨µ¨¹Ì´¼£¬ºúÂܲ·ËØ£©³ÉÑΣ¬È黯³¦Ç»ÄÚÓÍÖ¬£¬Ôö¼ÓÖ¬·¾Ã¸×÷ÓÃλµã£¬±ãÓÚÓÍÖ¬Ïû»¯ÎüÊÕ¡£
2¡¢ Àà¹Ì´¼¼¤ËØ
£¨1£©ÉöÉÏÏÙƤÖʼ¤ËØ£¨£·ÖÖ£©
£¨2£©ÐÔ¼¤ËØ
ÐÛÐÔ¼¤ËØ£ºØºÍèͪ
´ÆÐÔ¼¤ËØ£º´Æ¶þ´¼¡¢»ÆÌåͪ