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‫ﺩﻭﺭﺓﺣﻤﺾ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ ﻭﻣﺮﻛﺐ ﺑﻴﺮﻭﻓﺎﺕ‬

‫ﺩﻳﻬﻴﺪﺭﻭﺟﻴﻨﻴﺰ‪9‬‬

‫ﻟﻠﻤﻮﺍﺩﺍﻟﻤﺴﺎﻋﺪﺓ ﺍﻹﺿﺎﻓﻴﺔ ﺍﻟﻤﺘﻌﻠﻘﺔ ﺑﻬﺬﺍ‬

‫ﺍﻟﻔﺼﻞ ‪،‬ﻳﺮﺟﻰ ﺯﻳﺎﺭﺓﺍﻟﻨﻘﻄﺔ‪.‬‬

‫ﻧﻈﺮﺓﻋﺎﻣﺔ ﻋﻠﻰ ﺍﻟﺪﻭﺭﺓ ‪I.‬‬

‫ﺗﻠﻌﺐﺩﻭﺭﺓ ﺣﻤﺾ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ )]ﺩﻭﺭﺓ ‪ [TCA‬ﻭﺗﺴﻤﻰ ﺃﻳﻀﺎً ﺩﻭﺭﺓ ﺣﻤﺾ ﺍﻟﺴﺘﺮﻳﻚ ‪ ،‬ﺃﻭ ﺩﻭﺭﺓ‬
‫ﻛﺮﻳﺒﺲ( ﻋﺪﺓ ﺃﺩﻭﺍﺭ ﻓﻲ ﻋﻤﻠﻴﺔ ﺍﻟﺘﻤﺜﻴﻞ ﺍﻟﻐﺬﺍﺉﻲ‪ .‬ﺇﻧﻪ ﺍﻟﻤﺴﺎﺭ ﺍﻷﺧﻴﺮ ﺣﻴﺚ ﺗﺘﻼﻗﻰ ﻋﻤﻠﻴﺔ‬
‫ﺍﻟﺘﻘﻮﻳﺾﺍﻟﺘﺄﻛﺴﺪﻱ ﻟﻠﻜﺮﺑﻮﻫﻴﺪﺭﺍﺕ ﻭﺍﻷﺣﻤﺎﺽ ﺍﻷﻣﻴﻨﻴﺔ ﻭﺍﻷﺣﻤﺎﺽ ﺍﻟﺪﻫﻨﻴﺔ ‪ ،‬ﺣﻴﺚ ﻳﺘﻢ‬
‫ﺗﺤﻮﻳﻞﻫﻴﺎﻛﻠﻬﺎ ﺍﻟﻜﺮﺑﻮﻧﻴﺔ ﺇﻟﻰ ﺛﺎﻧﻲ ﺃﻛﺴﻴﺪ ﺍﻟﻜﺮﺑﻮﻥ )‪ ، (2.(CO‬ﻛﻤﺎ ﻫﻮ ﻣﻮﺿﺢ ﻓﻲ‬
‫ﺍﻟﺸﻜﻞ‪ .9.1‬ﺗﻮﻓﺮ ﻫﺬﻩ ﺍﻷﻛﺴﺪﺓ ﺍﻟﻄﺎﻗﺔ ﻹﻧﺘﺎﺝ ﻏﺎﻟﺒﻴﺔ ‪ ATP‬ﻓﻲ ﻣﻌﻈﻢ ﺍﻟﺤﻴﻮﺍﻧﺎﺕ ‪ ،‬ﺑﻤﺎ ﻓﻲ ﺫﻟﻚ‬
‫ﺍﻟﺒﺸﺮ‪.‬ﻧﻈﺮﺍً ﻷﻥ ﺩﻭﺭﺓ ‪ TCA‬ﺗﺤﺪﺙ ﺗﻤﺎﻣﺎً ﻓﻲ ﺍﻟﻤﻴﺘﻮﻛﻮﻧﺪﺭﻳﺎ ‪ ،‬ﻓﻬﻲ ﻗﺮﻳﺒﺔ ﺟﺪﺍً ﻣﻦ ﺳﻠﺴﻠﺔ ﻧﻘﻞ‬
‫ﺍﻹﻟﻜﺘﺮﻭﻥ)]‪ [ETC‬ﺍﻧﻈﺮ ﺹ ‪ ، (73‬ﻭﺍﻟﺘﻲ ﺗﻌﻤﻞ ﻋﻠﻰ ﺃﻛﺴﺪﺓ ﺍﻹﻧﺰﻳﻤﺎﺕ ﺍﻟﻤﺴﺎﻋﺪﺓ ﺍﻟﻤﺨﺘﺼﺮﺓ‬
‫ﻧﻴﻜﻮﺗﻴﻨﺎﻣﻴﺪﺍﻷﺩﻳﻨﻴﻦ ﺛﻨﺎﺉﻲ ﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪ )‪ (NADH‬ﻭﻓﻼﻓﻴﻦ ﺍﻷﺩﻳﻨﻴﻦ ﺛﻨﺎﺉﻲ ﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪ‬
‫)‪ (2(FADH‬ﺍﻟﺘﻲ ﺗﻨﺘﺠﻬﺎ‬
‫ﺩﻭﺭﺓ‪.‬ﺩﻭﺭﺓ ‪ TCA‬ﻫﻲ ﻣﺴﺎﺭ ﻫﻮﺍﺉﻲ ‪ ،‬ﻷﻥ ﺍﻷﻛﺴﺠﻴﻦ )‪ (2O‬ﻣﻄﻠﻮﺏ ﻛـ‬
‫ﻣﺘﻘﺒﻞﺍﻹﻟﻜﺘﺮﻭﻥ ﺍﻟﻨﻬﺎﺉﻲ‪ .‬ﺭﺩﻭﺩ ﺍﻟﻔﻌﻞ ﻣﺜﻞ ﻫﺪﻡ ﺑﻌﺾ ﺍﻷﺣﻤﺎﺽ ﺍﻷﻣﻴﻨﻴﺔ ﺗﻮﻟﺪ ﻣﻮﺍﺩ ﻭﺳﻴﻄﺔ‬
‫ﻣﻦﺍﻟﺪﻭﺭﺓ ﻭﺗﺴﻤﻰ ﺗﻔﺎﻋﻼﺕ ﻏﻴﺮ ﻗﺎﺑﻠﺔ ﻟﻠﺘﺼﻠﺐ )ﻣﻦ ﺍﻟﻴﻮﻧﺎﻧﻴﺔ ﺗﻌﻨﻲ "ﻣﻞء"(‪ .‬ﺗﻮﻓﺮ ﺩﻭﺭﺓ ‪TCA‬‬
‫ﺃﻳﻀﺎًﻣﻮﺍﺩ ﻭﺳﻴﻄﺔ ﻟﻌﺪﺩ ﻣﻦ ﺍﻟﺘﻔﺎﻋﻼﺕ ﺍﻻﺑﺘﻨﺎﺉﻴﺔ ﺍﻟﻤﻬﻤﺔ ‪ ،‬ﻣﺜﻞ ﺗﻜﻮﻳﻦ ﺍﻟﺠﻠﻮﻛﻮﺯ ﻣﻦ ﺍﻟﻬﻴﺎﻛﻞ‬
‫ﺍﻟﻜﺮﺑﻮﻧﻴﺔﻟﺒﻌﺾ ﺍﻷﺣﻤﺎﺽ ﺍﻷﻣﻴﻨﻴﺔ ﻭﺗﻮﻟﻴﻒ ﺑﻌﺾ ﺍﻷﺣﻤﺎﺽ ﺍﻷﻣﻴﻨﻴﺔ )ﺍﻧﻈﺮ ﺹ ‪ (267‬ﻭﺍﻟﻬﻴﻢ )‬
‫ﺍﻧﻈﺮﺹ ‪ .(278‬ﻟﺬﻟﻚ ‪ ،‬ﻻ ﻳﻨﺒﻐﻲ ﺍﻟﻨﻈﺮ ﺇﻟﻰ ﻫﺬﻩ ﺍﻟﺪﻭﺭﺓ ﻋﻠﻰ ﺃﻧﻬﺎ ﻧﻈﺎﻡ ﻣﻐﻠﻖ ‪ ،‬ﻭﻟﻜﻦ ‪ ،‬ﺑﺪﻻ ًﻣﻦ‬
‫ﺫﻟﻚ ‪،‬ﻛﻨﻈﺎﻡ ﻣﻔﺘﻮﺡ ﻣﻊ ﺩﺧﻮﻝ ﺍﻟﻤﺮﻛﺒﺎﺕ ﻭﺗﺮﻛﻬﺎ ﻛﻤﺎ ﻫﻮ ﻣﻄﻠﻮﺏ‪.‬‬
 ‫ﺍﻟﺸﻜﻞ‪ 9.1‬ﺗﻈﻬﺮ ﺩﻭﺭﺓ ﺣﻤﺾ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻠﻴﻚ ﻛﺠﺰء ﻣﻦ ﺍﻟﻤﺴﺎﺭﺍﺕ ﺍﻷﺳﺎﺳﻴﺔ ﻻﺳﺘﻘﻼﺏ‬
‫ﺍﻟﻄﺎﻗﺔ‪].‬ﻣﻼﺣﻈﺔ‪ :‬ﺍﻧﻈﺮﺍﻟﺸﻜﻞ ‪ ،8.2‬ﺹ‪ 92 .‬ﻟﻠﺤﺼﻮﻝ ﻋﻠﻰ ﺧﺮﻳﻄﺔ ﺃﻛﺜﺮ ﺗﻔﺼﻴﻼ ﻟﻌﻤﻠﻴﺔ ﺍﻟﺘﻤﺜﻴﻞ‬
‫ﺍﻟﻐﺬﺍﺉﻲ‪ =2CO [.‬ﺛﺎﻧﻲ ﺃﻛﺴﻴﺪ ﺍﻟﻜﺮﺑﻮﻥ ‪ = CoA‬ﺃﻧﺰﻳﻢ ﺃ‪.‬‬

‫ﺗﻔﺎﻋﻼﺕﺍﻟﺪﻭﺭﺓ ‪II.‬‬
‫ﻓﻲﺩﻭﺭﺓ ‪ ، TCA‬ﻳﺘﻢ ﺗﻜﺜﻴﻒ ﺃﻭﻛﺴﺎﻟﻮ ﺃﺳﻴﺘﺎﺕ )‪ (OAA‬ﺃﻭﻻ ًﻣﻊ ﻣﺠﻤﻮﻋﺔ ﺃﺳﻴﺘﻴﻞ ﻣﻦ ﺃﻧﺰﻳﻢ‬
‫ﺃﺳﻴﺘﻴﻞﺃ )‪ (CoA‬ﺛﻢ ﻳﺘﻢ ﺗﺠﺪﻳﺪﻩ ﻋﻨﺪ ﺍﻛﺘﻤﺎﻝ ﺍﻟﺪﻭﺭﺓ )ﺍﻧﻈﺮﺍﻟﺸﻜﻞ ‪ .(9.1‬ﻳﺪﺧﻞ ﺍﺛﻨﺎﻥ ﻣﻦ‬
‫ﺍﻟﻜﺎﺭﺑﻮﻧﺎﺕﺇﻟﻰ ﺍﻟﺪﻭﺭﺓ ﻓﻲ ﺻﻮﺭﺓ ﺃﺳﻴﺘﻴﻞ ‪ CoA‬ﻭﻳﻐﺎﺩﺭ ﺍﺛﻨﺎﻥ ﻓﻲ ﺻﻮﺭﺓ ﺃﻭﻝ ﺃﻛﺴﻴﺪ ﺍﻟﻜﺮﺑﻮﻥ‪.2‬‬
‫ﻟﺬﻟﻚ ‪،‬ﻻ ﻳﺆﺩﻱ ﺩﺧﻮﻝ ﺣﻤﺾ ﺃﺳﻴﺘﻴﻞ ﻭﺍﺣﺪ ﻓﻲ ﺟﻮﻟﺔ ﻭﺍﺣﺪﺓ ﻣﻦ ﺩﻭﺭﺓ ‪ TCA‬ﺇﻟﻰ ﺻﺎﻓﻲ ﺇﻧﺘﺎﺝ ﺃﻭ‬
‫ﺍﺳﺘﻬﻼﻙﺍﻟﻤﻮﺍﺩ ﺍﻟﻮﺳﻴﻄﺔ‪.‬‬

‫‪ CoA‬ﺇﻧﺘﺎﺝ ﺍﻷﺳﻴﺘﻴﻞ ‪A.‬‬

‫ﺍﻟﻤﺼﺪﺭﺍﻟﺮﺉﻴﺴﻲ ﻷﺳﻴﺘﻴﻞ ‪ CoA‬ﻟﺪﻭﺭﺓ ‪ TCA‬ﻫﻮ ﻧﺰﻉ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ ﺍﻟﻤﺆﻛﺴﺪ ﻣﻦ‬
‫ﺍﻟﺒﻴﺮﻭﻓﺎﺕﺑﻮﺍﺳﻄﺔ ﺍﻹﻧﺰﻳﻢ ﺍﻟﻤﺘﻌﺪﺩﻣﺮﻛﺐ ﻧﺎﺯﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻦ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ)ﻣﺠﻤﻊ ‪ ،PDH‬ﺃﻭ‬
‫‪ .(PDHC‬ﻭﻣﻊ ﺫﻟﻚ ‪ ،‬ﻓﺈﻥ‪)PDHC‬ﺍﻟﻤﻮﺻﻮﻑ ﺃﺩﻧﺎﻩ( ﻟﻴﺲ ﺃﺣﺪ ﻣﻜﻮﻧﺎﺕ ﺩﻭﺭﺓ ‪ .TCA‬ﻳﺘﻢ‬
‫ﻧﻘﻞﺍﻟﺒﻴﺮﻭﻓﺎﺕ ‪ ،‬ﺍﻟﻤﻨﺘﺞ ﺍﻟﻨﻬﺎﺉﻲ ﻟﺘﺤﻠﻞ ﺍﻟﺴﻜﺮ ﺍﻟﻬﻮﺍﺉﻲ ‪ ،‬ﻣﻦ ﺍﻟﻌﺼﺎﺭﺓ ﺍﻟﺨﻠﻮﻳﺔ ﺇﻟﻰ‬
‫ﻣﺼﻔﻮﻓﺔﺍﻟﻤﻴﺘﻮﻛﻮﻧﺪﺭﻳﺎ ﺑﻮﺍﺳﻄﺔ ﺣﺎﻣﻞ ﻣﻴﺘﻮﻛﻮﻧﺪﺭﻳﺎ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ ﻟﻠﻐﺸﺎء ﺍﻟﺪﺍﺧﻠﻲ‬
‫ﻟﻠﻤﻴﺘﻮﻛﻮﻧﺪﺭﻳﺎ‪.‬ﻓﻲ ﺍﻟﻤﺼﻔﻮﻓﺔ ‪ ،‬ﻓﺈﻥ ﻣﻠﻒ‪PDHC‬ﻳﺤﻮﻝ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ ﺇﻟﻰ ﺃﺳﻴﺘﻴﻞ ‪] .CoA‬‬
‫ﻣﻼﺣﻈﺔ‪:‬ﺃﻛﺴﺪﺓ ﺍﻷﺣﻤﺎﺽ ﺍﻟﺪﻫﻨﻴﺔ ﻫﻲ ﻣﺼﺪﺭ ﺁﺧﺮ ﻷﺳﻴﺘﻴﻞ ‪) CoA‬ﺍﻧﻈﺮ ﺹ ‪[.(192‬‬

‫‪.1‬ﺇﻧﺰﻳﻤﺎﺕ ﻣﻜﻮﻥ ‪ :PDHC‬ﺇﻥ‪PDHC‬ﻋﺒﺎﺭﺓ ﻋﻦ ﺗﺠﻤﻊ ﺑﺮﻭﺗﻴﻨﻲ ﻣﻦ ﻧﺴﺦ ﻣﺘﻌﺪﺩﺓ ﻣﻦ ﺛﻼﺛﺔ‬

‫ﺇﻧﺰﻳﻤﺎﺕ ‪،‬ﺑﻴﺮﻭﻓﺎﺕ ﺩﻳﻜﺎﺭﺑﻮﻛﺴﻴﻼﺯ)]ﻩ ‪ [1‬ﺍﺗﺼﻠﺖ ﻓﻲ ﺑﻌﺾ ﺍﻷﺣﻴﺎﻥﺑﻴﺮﻭﻓﺎﺕ‬
‫ﺩﻳﻬﻴﺪﺭﻭﺟﻴﻨﻴﺰ( ‪،‬ﺛﻨﺎﺉﻲ ﻫﻴﺪﺭﻭﻟﻴﺒﻮﻳﻞ ﺗﺮﺍﻧﺲ ﺃﺳﻴﺘﻴﻼﺯ )ﻩ ‪ ،(2‬ﻭﺩﻳﻬﻴﺪﺭﻭﻟﻴﺒﻮﻳﻞ‬
‫ﺩﻳﻬﻴﺪﺭﻭﺟﻴﻨﻴﺰ)ﻩ ‪ .(3‬ﻛﻞ ﻣﻨﻬﺎ ﻳﺤﻔﺰ ﺟﺰءﺍً ﻣﻦ ﺍﻟﺘﻔﺎﻋﻞ ﺍﻟﻜﻠﻲ )ﺍﻟﺸﻜﻞ ‪ .(9.2‬ﻳﺮﺑﻂ‬
‫ﺍﺭﺗﺒﺎﻃﻬﻢﺍﻟﻤﺎﺩﻱ ﺑﻴﻦ ﺭﺩﻭﺩ ﺍﻟﻔﻌﻞ ﻓﻲ ﺗﺴﻠﺴﻞ ﻣﻨﺎﺳﺐ ﺩﻭﻥ ﺇﻃﻼﻕ ﺍﻟﻮﺳﻄﺎء‪.‬‬
‫ﺑﺎﻹﺿﺎﻓﺔﺇﻟﻰ ﺍﻹﻧﺰﻳﻤﺎﺕ ﺍﻟﻤﺸﺎﺭﻛﺔ ﻓﻲ ﺗﺤﻮﻳﻞ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ ﺇﻟﻰ ﺃﺳﻴﺘﻴﻞ ‪ ، CoA‬ﻓﺈﻥ‬
‫‪PDHC‬ﻳﺤﺘﻮﻱ ﺃﻳﻀﺎً ﻋﻠﻰ ﺍﺛﻨﻴﻦ ﻣﻦ ﺍﻹﻧﺰﻳﻤﺎﺕ ﺍﻟﺘﻨﻈﻴﻤﻴﺔ ‪،‬ﺑﻴﺮﻭﻓﺎﺕ ﺩﻳﻬﻴﺪﺭﻭﺟﻴﻨﻴﺰ‬
‫ﻛﻴﻨﺎﺯ)ﻛﻴﻨﺎﺯ ‪ (PDH‬ﻭﺑﻴﺮﻭﻓﺎﺕ ﺩﻳﻬﻴﺪﺭﻭﺟﻴﻨﻴﺰ ﻓﻮﺳﻔﺎﺗﻴﺰ)ﺇﻧﺰﻳﻢ ‪ PDH‬ﺍﻟﻔﻮﺳﻔﺎﺗﻴﺰ(‪.‬‬
 ‫ﺍﻟﺸﻜﻞ‪ 9.2‬ﺁﻟﻴﺔ ﻋﻤﻞ ﺍﻹﻧﺰﻳﻤﺎﺕ )ﻩ( ﺍﻟﺘﺎﺑﻊﻣﺮﻛﺐ ﻧﺎﺯﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻦ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ‪] .‬ﻣﻼﺣﻈﺔ‪ :‬ﺟﻤﻴﻊ‬
‫ﺍﻹﻧﺰﻳﻤﺎﺕﺍﻟﻤﺴﺎﻋﺪﺓ ﻟﻠﻤﺮﻛﺐ ‪ ،‬ﺑﺎﺳﺘﺜﻨﺎء ﺣﻤﺾ ﻟﻴﺒﻮﻳﻚ ‪ ،‬ﻣﺸﺘﻘﺔ ﻣﻦ ﺍﻟﻔﻴﺘﺎﻣﻴﻨﺎﺕ‪ .‬ﻳﺘﻜﻮﻥ‬
‫‪ TPP‬ﻣﻦ ﺍﻟﺜﻴﺎﻣﻴﻦ ‪ ،‬ﻭ ‪ FAD‬ﻣﻦ ﺍﻟﺮﻳﺒﻮﻓﻼﻓﻴﻦ ‪ ،‬ﻭ ‪ NAD‬ﻣﻦ ﺍﻟﻨﻴﺎﺳﻴﻦ ‪ ،‬ﻭ ‪ CoA‬ﻣﻦ ﺣﻤﺾ‬
‫ﺍﻟﺒﺎﻧﺘﻮﺛﻨﻴﻚ‪ =2CO [.‬ﻛﺮﺑﻮﻥ‬
‫ﺛﺎﻧﻲﺃﻛﺴﻴﺪ؛ ‪ = TPP‬ﺑﻴﺮﻭﻓﻮﺳﻔﺎﺕ ﺍﻟﺜﻴﺎﻣﻴﻦ ؛ ‪ = L‬ﺣﻤﺾ ﻟﻴﺒﻮﻳﻚ‪ = CoA .‬ﺃﻧﺰﻳﻢ ‪ A‬؛ ‪(2FAD )H‬‬
‫ﻭ (‪ = NAD )H‬ﺍﻟﻔﻼﻓﻴﻦ ﻭﺍﻟﻨﻴﻜﻮﺗﻴﻨﺎﻣﻴﺪ ﺍﻷﺩﻳﻨﻴﻦ ﺛﻨﺎﺉﻲ ﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪﺍﺕ ؛ ~ =‬
‫ﺭﺍﺑﻄﺔﻋﺎﻟﻴﺔ ﺍﻟﻄﺎﻗﺔ‪.‬‬

‫‪.2‬ﺍﻹﻧﺰﻳﻤﺎﺕ ﺍﻟﻤﺴﺎﻋﺪﺓ‪ :‬ﺇﻥ‪PDHC‬ﻳﺤﺘﻮﻱ ﻋﻠﻰ ﺧﻤﺴﺔ ﺃﻧﺰﻳﻤﺎﺕ ﻣﺴﺎﻋﺪﺓ ﺗﻌﻤﻞ ﻛﻨﺎﻗﻼﺕ‬

‫ﺃﻭﻣﺆﻛﺴﺪﺍﺕ ﻟﻠﻮﺳﻄﺎء ﻟﻠﺘﻔﺎﻋﻼﺕ ﺍﻟﻤﻮﺿﺤﺔ ﻓﻲﺍﻟﺸﻜﻞ ‪.9.2‬ﻩ ‪ 1‬ﻳﺘﻄﻠﺐ‬
‫ﺑﻴﺮﻭﻓﻮﺳﻔﺎﺕﺍﻟﺜﻴﺎﻣﻴﻦ )‪، (TPP‬ﻩ ‪2‬ﻳﺘﻄﻠﺐ ﺣﻤﺾ ﻟﻴﺒﻮﻳﻚ ﻭ ‪، CoA‬‬
‫ﻭﻩ ‪3‬ﻳﺘﻄﻠﺐ ‪ FAD‬ﻭ ‪] .+NAD‬ﻣﻼﺣﻈﺔ‪ :‬ﻳﺮﺗﺒﻂ ‪ ، TPP‬ﻭﺣﻤﺾ ﺍﻟﻠﻴﺒﻮﻳﻚ ‪ ،‬ﻭ ‪FAD‬‬
‫ﺍﺭﺗﺒﺎﻃﺎًﻭﺛﻴﻘﺎً ﺑﺎﻹﻧﺰﻳﻤﺎﺕ ﻭﻳﻌﻤﻠﻮﻥ ﻛﺄﻧﺰﻳﻤﺎﺕ ﻣﺴﺎﻋﺪﺓ ‪ -‬ﻣﺠﻤﻮﻋﺎﺕ ﺍﺻﻄﻨﺎﻋﻴﺔ )ﺍﻧﻈﺮ‬
‫ﺹ‪[.(54‬‬

‫ﻳﻤﻜﻦﺃﻥ ﻳﺴﺒﺐ ﻧﻘﺺ ﺍﻟﺜﻴﺎﻣﻴﻦ ﺃﻭ ﺍﻟﻨﻴﺎﺳﻴﻦ ﻣﺸﺎﻛﻞ ﺧﻄﻴﺮﺓ ﻓﻲ ﺍﻟﺠﻬﺎﺯ ﺍﻟﻌﺼﺒﻲ‬

‫ﺍﻟﻤﺮﻛﺰﻱ‪.‬ﻭﺫﻟﻚ ﻷﻥ ﺧﻼﻳﺎ ﺍﻟﺪﻣﺎﻍ ﻏﻴﺮ ﻗﺎﺩﺭﺓ ﻋﻠﻰ ﺇﻧﺘﺎﺝ ﻣﺎ ﻳﻜﻔﻲ ﻣﻦ ‪) ATP‬ﻋﺒﺮ ﺩﻭﺭﺓ ‪(TCA‬‬
‫ﺇﺫﺍﻛﺎﻥ‪PDHC‬ﻏﻴﺮ ﻧﺸﻂ‪ .‬ﻳﻤﻜﻦ ﺭﺅﻳﺔ ‪ ، Wernicke-Korsakoff‬ﻭﻫﻮ ﻣﺘﻼﺯﻣﺔ ﺍﻟﺬﻫﺎﻥ‬
‫ﺍﻟﺪﻣﺎﻏﻲﺑﺴﺒﺐ ﻧﻘﺺ ﺍﻟﺜﻴﺎﻣﻴﻦ ‪ ،‬ﻣﻊ ﺗﻌﺎﻃﻲ ﺍﻟﻜﺤﻮﻝ )ﺍﻧﻈﺮ ﺹ ‪.(383‬‬

‫‪.3‬ﺍﻟﻼﺉﺤﺔ‪ :‬ﺍﻟﺘﻌﺪﻳﻼﺕ ﺍﻟﺘﺴﺎﻫﻤﻴﺔ ﻣﻦ ﻗﺒﻞ ﺍﺛﻨﻴﻦ ﻣﻦ ﺍﻹﻧﺰﻳﻤﺎﺕ ﺍﻟﺘﻨﻈﻴﻤﻴﺔ ‪PDHC‬‬

‫ﺍﻟﺘﻨﺸﻴﻂﻭﺇﻟﻐﺎء ﺍﻟﺘﻨﺸﻴﻂ ﺑﺎﻟﺘﻨﺎﻭﺏﻩ ‪.1‬ﻛﻴﻨﺎﺯ ‪ PDH‬ﺍﻟﻔﻮﺳﻔﻮﺭﻳﻼﺕ ﻭﻳﺜﺒﻂ ﻧﺸﺎﻃﻬﺎﻩ ‪،1‬‬
‫ﺑﻴﻨﻤﺎﺇﻧﺰﻳﻢ ‪ PDH‬ﺍﻟﻔﻮﺳﻔﺎﺗﻴﺰ ﻳﻨﺸﻂ ﻭﻳﻨﺸﻂﻩ ‪)1‬ﺍﻟﺸﻜﻞ ‪ .(9.3‬ﺍﻝﻛﻴﻨﺎﺯﻫﻮ ﻧﻔﺴﻪ‬
 ‫ﻳﺘﻢﺗﻨﺸﻴﻄﻪ ﺑﻮﺍﺳﻄﺔ ‪ ATP‬ﻭ ‪ acetyl CoA‬ﻭ ‪ .NADH‬ﻟﺬﻟﻚ ‪ ،‬ﻓﻲ ﻭﺟﻮﺩ ﻫﺬﻩ‬
‫ﺍﻟﻤﻨﺘﺠﺎﺕﻋﺎﻟﻴﺔ ﺍﻟﻄﺎﻗﺔ ‪ ،‬ﻓﺈﻥ‪PDHC‬ﻣﺘﻮﻗﻒ‪] .‬ﻣﻼﺣﻈﺔ‪ :‬ﺇﻧﻪ ﻓﻲ ﺍﻟﻮﺍﻗﻊ ﺍﺭﺗﻔﺎﻉ ﻓﻲ ‪ADP‬‬
‫‪) ATP /‬ﺛﻨﺎﺉﻲ ﻓﻮﺳﻔﺎﺕ ﺍﻷﺩﻳﻨﻮﺯﻳﻦ( ‪،‬‬
‫‪ ،+NADH / NAD‬ﺃﻭ ﻧﺴﺐ ﺍﻷﺳﻴﺘﻴﻞ ‪ CoA / CoA‬ﺍﻟﺘﻲ ﺗﺆﺛﺮ ﻋﻠﻰ ﺍﻟﻨﺸﺎﻁ ﺍﻹﻧﺰﻳﻤﻲ‪[.‬‬
‫ﺍﻟﺒﻴﺮﻭﻓﺎﺕﻫﻮ ﻣﺜﺒﻂ ﻗﻮﻱ ﻟـﻛﻴﻨﺎﺯ ‪ .PDH‬ﻟﺬﻟﻚ ‪ ،‬ﺇﺫﺍ ﻛﺎﻥ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ‬
‫ﺗﺮﻛﻴﺰﺍﺕﻣﺮﺗﻔﻌﺔ ‪،‬ﻩ ‪1‬ﺳﺘﻜﻮﻥ ﻧﺸﻄﺔ ﺇﻟﻰ ﺃﻗﺼﻰ ﺣﺪ‪ .‬ﺍﻟﻜﺎﻟﺴﻴﻮﻡ )‪ (+2Ca‬ﻫﻮ ﻣﻨﺸﻂ‬
‫ﻗﻮﻱﻟـﺇﻧﺰﻳﻢ ‪ PDH‬ﺍﻟﻔﻮﺳﻔﺎﺗﻴﺰ‪ ،‬ﺗﻨﺸﻴﻂﻩ ‪1‬ﻧﺸﺎﻁ‪ .‬ﻫﺬﻩ‬
‫ﻣﻬﻢﺑﺸﻜﻞ ﺧﺎﺹ ﻓﻲ ﺍﻟﻌﻀﻼﺕ ﺍﻟﻬﻴﻜﻠﻴﺔ ‪ ،‬ﺣﻴﺚ ‪+2Ca‬ﺍﻹﻓﺮﺍﺝ ﺃﺛﻨﺎء ﺍﻻﻧﻘﺒﺎﺽ ﻳﺤﻔﺰ‬
‫‪PDHC‬ﻭﺑﺎﻟﺘﺎﻟﻲ ﺇﻧﺘﺎﺝ ﺍﻟﻄﺎﻗﺔ‪] .‬ﻣﻼﺣﻈﺔ‪ :‬ﻋﻠﻰ ﺍﻟﺮﻏﻢ ﻣﻦ ﺃﻥ ﺍﻟﺘﻨﻈﻴﻢ ﺍﻟﺘﺴﺎﻫﻤﻲ ﻣﻦ‬
‫ﻗﺒﻞﻛﻴﻨﺎﺯﻭﺍﻟﻔﻮﺳﻔﺎﺗﻴﺰﻫﻮ ﺍﻷﺳﺎﺳﻲ ‪ ،‬ﻭ‪PDHC‬ﻳﺨﻀﻊ ﺃﻳﻀﺎً ﻟﺘﺜﺒﻴﻂ ﺍﻟﻤﻨﺘﺞ )‪ NADH‬ﻭ‬
‫‪[.(acetyl CoA‬‬
‫ﺍﻟﺸﻜﻞ‪ 9.3‬ﺗﻨﻈﻴﻢﺑﻴﺮﻭﻓﺎﺕ ﺩﻳﻬﻴﺪﺭﻭﺟﻴﻨﻴﺰ)‪(PDH‬ﻣﺮﻛﺐ‪ = .‬ﺍﻟﻔﻮﺳﻔﺎﺕ ]ﻳﺸﻴﺮ ﺇﻟﻰ ﺗﺜﺒﻴﻂ ﺍﻟﻤﻨﺘﺞ‬
‫‪[.‬‬

‫‪.4‬ﺍﻟﻨﻘﺺ‪ :‬ﻧﻘﺺ ﺍﻟﻮﺣﺪﺍﺕ ﺍﻟﻔﺮﻋﻴﺔ ‪ α‬ﻟﻠﺮﺑﺎﻋﻲﻩ ‪ 1‬ﻣﻜﻮﻥ ﻣﻦ‪PDHC‬ﻋﻠﻰ ﺍﻟﺮﻏﻢ ﻣﻦ ﻧﺪﺭﺗﻪ‬

‫ﺍﻟﺸﺪﻳﺪﺓ ‪،‬ﻓﻬﻮ ﺍﻟﺴﺒﺐ ﺍﻟﻜﻴﻤﻴﺎﺉﻲ ﺍﻟﺤﻴﻮﻱ ﺍﻷﻛﺜﺮ ﺷﻴﻮﻋﺎً ﻟﻠﺤﻤﺎﺽ ﺍﻟﻠﺒﻨﻲ ﺍﻟﺨﻠﻘﻲ‪.‬‬
‫ﻳﺆﺩﻱﺍﻟﻨﻘﺺ ﺇﻟﻰ ﺍﻧﺨﻔﺎﺽ ﺍﻟﻘﺪﺭﺓ ﻋﻠﻰ ﺗﺤﻮﻳﻞ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ ﺇﻟﻰ ﺃﺳﻴﺘﻴﻞ ‪ ، CoA‬ﻣﻤﺎ‬
‫ﻳﺘﺴﺒﺐﻓﻲ ﺗﺤﻮﻳﻞ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ ﺇﻟﻰ ﺍﻟﻼﻛﺘﺎﺕ ﻋﺒﺮﻧﺎﺯﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻦ ﺍﻟﻼﻛﺘﺎﺕ)ﺍﻧﻈﺮ ﺹ‬
‫‪ .(103‬ﻫﺬﺍ ﻳﺨﻠﻖ ﻣﺸﺎﻛﻞ ﺧﺎﺻﺔ ﻟﻠﺪﻣﺎﻍ ‪ ،‬ﻭﺍﻟﺘﻲ ﺗﻌﺘﻤﺪ ﻋﻠﻰ ﺩﻭﺭﺓ ‪TCA‬‬
 ‫ﻟﻤﻌﻈﻢﻃﺎﻗﺘﻪ ﻭﻫﻮ ﺣﺴﺎﺱ ﺑﺸﻜﻞ ﺧﺎﺹ ﻟﻠﺤﻤﺎﺽ‪ .‬ﺍﻷﻋﺮﺍﺽ ﻣﺘﻐﻴﺮﺓ ﻭﺗﺸﻤﻞ‬
‫ﺍﻟﺘﻨﻜﺲﺍﻟﻌﺼﺒﻲ ‪ ،‬ﻭﺍﻟﺘﺸﻨﺞ ﺍﻟﻌﻀﻠﻲ ‪ ،‬ﻭﻓﻲ ﺷﻜﻞ ﺑﺪﺍﻳﺔ ﺣﺪﻳﺜﻲ ﺍﻟﻮﻻﺩﺓ ‪ ،‬ﺍﻟﻤﻮﺕ‬
‫ﺍﻟﻤﺒﻜﺮ‪.‬ﻳﺮﺗﺒﻂ ﺟﻴﻦ ﺍﻟﻮﺣﺪﺓ ﺍﻟﻔﺮﻋﻴﺔ ‪ α‬ﺑـ ‪ ، X‬ﻭﻷﻥ ﻛﻼ ﻣﻦ ﺍﻟﺬﻛﻮﺭ ﻭﺍﻹﻧﺎﺙ ﻗﺪ ﻳﺘﺄﺛﺮﻭﻥ ‪،‬‬
‫ﻳﺘﻢﺗﺼﻨﻴﻒ ﺍﻟﻨﻘﺺ ﻋﻠﻰ ﺃﻧﻪ ﺳﺎﺉﺪ ﻣﺮﺗﺒﻂ ﺑـ ‪ .X‬ﻋﻠﻰ ﺍﻟﺮﻏﻢ ﻣﻦ ﻋﺪﻡ ﻭﺟﻮﺩ ﻋﻼﺝ‬
‫ﻣﺜﺒﺖﻝ‪PDHC‬ﻗﺪ ﻳﺆﺩﻱ ﻧﻘﺺ ﺍﻟﻜﺮﺑﻮﻫﻴﺪﺭﺍﺕ ﻭﺍﻟﻘﻴﻮﺩ ﺍﻟﻐﺬﺍﺉﻴﺔ ﻭﺗﻨﺎﻭﻝ ﺍﻟﺜﻴﺎﻣﻴﻦ ﺇﻟﻰ‬
‫ﺗﻘﻠﻴﻞﺍﻷﻋﺮﺍﺽ ﻟﺪﻯ ﻣﺮﺿﻰ ﻣﻌﻴﻨﻴﻦ‪.‬‬

‫ﻣﺘﻼﺯﻣﺔﻟﻲ )ﺍﻋﺘﻼﻝ ﺍﻟﺪﻣﺎﻍ ﺍﻟﻨﺨﺎﻋﻲ ﺗﺤﺖ ﺍﻟﺤﺎﺩ ﺗﺤﺖ ﺍﻟﺤﺎﺩ( ﻫﻮ ﺍﺿﻄﺮﺍﺏ ﺗﻨﻜﺴﻲ‬
‫ﻋﺼﺒﻲﻧﺎﺩﺭ ﻭﺗﻘﺪﻣﻲ ﻧﺎﺟﻢ ﻋﻦ ﻋﻴﻮﺏ ﻓﻲ ﺇﻧﺘﺎﺝ ﺍﻟـ ‪ ATP‬ﻓﻲ ﺍﻟﻤﻴﺘﻮﻛﻮﻧﺪﺭﻳﺎ ‪ ،‬ﻭﺫﻟﻚ ﻓﻲ‬
‫ﺍﻟﻤﻘﺎﻡﺍﻷﻭﻝ ﻧﺘﻴﺠﺔ ﺍﻟﻄﻔﺮﺍﺕ ﻓﻲ ﺍﻟﺠﻴﻨﺎﺕ ﺍﻟﺘﻲ ﺗﺸﻔﺮ ﺑﺮﻭﺗﻴﻨﺎﺕ‪PDHC‬ﺃﻭ ‪ ETC‬ﺃﻭ‬
‫ﺳﻴﻨﺴﻴﺰ‪ .ATP‬ﻳﻤﻜﻦ ﺃﻥ ﻳﺘﺄﺛﺮ ﻛﻞ ﻣﻦ ﺍﻟﺤﻤﺾ ﺍﻟﻨﻮﻭﻱ ﻭﺍﻟﻤﻴﺘﻮﻛﻮﻧﺪﺭﻳﺎ‪.‬‬

‫‪.5‬ﺍﻟﺘﺴﻤﻢ ﺑﺎﻟﺰﺭﻧﻴﺦ‪ :‬ﻛﻤﺎ ﺳﺒﻖ ﻭﺻﻔﻪ )ﺍﻧﻈﺮ ﺹ ‪ ، (101‬ﻳﻤﻜﻦ ﺃﻥ ﻳﺘﺪﺍﺧﻞ ﺍﻟﺰﺭﻧﻴﺦ‬

‫ﺧﻤﺎﺳﻲﺍﻟﺘﻜﺎﻓﺆ )ﺍﻟﺰﺭﻧﻴﺦ( ﻣﻊ ﺗﺤﻠﻞ ﺍﻟﺴﻜﺮ ﻓﻲﺟﻠﻴﺴﺮﺍﻟﺪﻫﻴﺪ ‪ -3‬ﺍﻟﻔﻮﺳﻔﺎﺕﺧﻄﻮﺓ ‪،‬‬
‫ﻭﺑﺎﻟﺘﺎﻟﻲﺗﻘﻠﻴﻞ ﺇﻧﺘﺎﺝ ‪ .ATP‬ﻭﻣﻊ ﺫﻟﻚ ‪ ،‬ﻓﺈﻥ ﺍﻟﺘﺴﻤﻢ ﺑﺎﻟﺰﺭﻧﻴﺦ ﻳﺮﺟﻊ ﻓﻲ ﺍﻟﻤﻘﺎﻡ ﺍﻷﻭﻝ‬
‫ﺇﻟﻰﺗﺜﺒﻴﻂ ﻣﻌﻘﺪﺍﺕ ﺍﻹﻧﺰﻳﻢ ﺍﻟﺘﻲ ﺗﺘﻄﻠﺐ ﺣﻤﺾ ﻟﻴﺒﻮﻳﻚ ﺑﺎﻋﺘﺒﺎﺭﻩ ﺃﻧﺰﻳﻤﺎً ‪ ،‬ﺑﻤﺎ ﻓﻲ ﺫﻟﻚ‬
‫‪PDH‬ﻭﻧﺎﺯﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻦ ﺃﻟﻔﺎ ﻛﻴﺘﻮﺟﻠﻮﺗﺎﺭﺍﺕ)ﺍﻧﻈﺮ ‪ .E‬ﺃﺩﻧﺎﻩ( ‪ ،‬ﻭﺳﻠﺴﻠﺔ ﻣﺘﻔﺮﻋﺔ ﻣﻦ‬
‫ﺣﻤﺾﺃﻟﻔﺎ ﻛﻴﺘﻮ ﺩﻳﻬﻴﺪﺭﻭﺟﻴﻨﻴﺰ)ﺍﻧﻈﺮ ﺹ ‪ .(266‬ﻳﺸﻜﻞ ﺍﻟﺰﺭﻧﻴﺦ )ﺍﻟﺸﻜﻞ ﺛﻼﺛﻲ ﺍﻟﺘﻜﺎﻓﺆ‬
‫ﻣﻦﺍﻟﺰﺭﻧﻴﺦ( ﻣﺮﻛﺒﺎً ﻣﺴﺘﻘﺮﺍً ﻣﻊ ﻣﺠﻤﻮﻋﺎﺕ ﺍﻟﺜﻴﻮﻝ )‪ (SH−‬ﻣﻦ ﺣﻤﺾ ﻟﻴﺒﻮﻳﻚ ‪ ،‬ﻣﻤﺎ‬
‫ﻳﺠﻌﻞﻫﺬﺍ ﺍﻟﻤﺮﻛﺐ ﻏﻴﺮ ﻣﺘﺎﺡ ﻟﻴﻜﻮﻥ ﺑﻤﺜﺎﺑﺔ ﺃﻧﺰﻳﻢ‪ .‬ﻋﻨﺪﻣﺎ ﻳﺮﺗﺒﻂ ﺑﺤﻤﺾ ﻟﻴﺒﻮﻳﻚ ﻓﻲ‬
‫‪ ،PDHC‬ﺍﻟﺒﻴﺮﻭﻓﺎﺕ )ﻭﺑﺎﻟﺘﺎﻟﻲ ﺍﻟﻼﻛﺘﺎﺕ(‪ .‬ﻛﻤﺎ ﻫﻮ ﺍﻟﺤﺎﻝ ﻣﻊ‪PDHC‬ﺍﻟﻨﻘﺺ ‪ ،‬ﻭﻫﺬﺍ ﻳﺆﺛﺮ‬
‫ﺑﺸﻜﻞﺧﺎﺹ ﻋﻠﻰ ﺍﻟﺪﻣﺎﻍ ‪ ،‬ﻭﻳﺴﺒﺐ ﺍﺿﻄﺮﺍﺑﺎﺕ ﻋﺼﺒﻴﺔ ﻭﺍﻟﻤﻮﺕ‪.‬‬

‫ﺗﺮﻛﻴﺐﺍﻟﺴﻴﺘﺮﺍﺕ‬
‫ﻳﺘﻢﺗﺤﻔﻴﺰ ﺍﻟﺘﻜﺜﻴﻒ ﻏﻴﺮ ﺍﻟﻘﺎﺑﻞ ﻟﻠﻌﻜﺲ ﻷﺳﻴﺘﻴﻞ ‪ CoA‬ﻭ ‪ OAA‬ﻟﺘﻜﻮﻳﻦ ﺳﺘﺮﺍﺕ )ﺣﻤﺾ‬
‫ﺛﻼﺛﻲﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ( ﺑﻮﺍﺳﻄﺔﺳﻴﻨﺜﺎﺱ ﺍﻟﺴﺘﺮﺍﺕ‪ ،‬ﺍﻹﻧﺰﻳﻢ ﺍﻟﺒﺎﺩﺉ ﻟﺪﻭﺭﺓ ‪) TCA‬ﺍﻟﺸﻜﻞ ‪.(9.4‬‬
‫ﻫﺬﺍﺍﻟﺘﻜﺜﻴﻒ ﺃﻟﺪﻭﻝ ﻟﻪ ﺳﻠﺒﻲ ﻟﻠﻐﺎﻳﺔ‬
‫ﺍﻟﺘﻐﻴﻴﺮﻓﻲ ﻣﻌﻴﺎﺭ ﺍﻟﻄﺎﻗﺔ ﺍﻟﺤﺮﺓ )]‪ [0G‬ﺍﻧﻈﺮ ﺹ‪ ، (70 .‬ﻭﺍﻟﺬﻱ ﻳﻔﻀﻞ ﺑﺸﺪﺓ ﺗﻜﻮﻳﻦ‬
‫ﺍﻟﺴﺘﺮﺍﺕ‪.‬ﻳﺘﻢ ﺗﺜﺒﻴﻂ ﺍﻹﻧﺰﻳﻢ ﺑﻮﺍﺳﻄﺔ ﺍﻟﺴﺘﺮﺍﺕ )ﺗﺜﺒﻴﻂ ﺍﻟﻤﻨﺘﺞ(‪ .‬ﺗﻮﺍﻓﺮ ﺍﻟﺮﻛﻴﺰﺓ ﻫﻮ ﻭﺳﻴﻠﺔ‬
‫ﺃﺧﺮﻯﻟﺘﻨﻈﻴﻢ ﻝﺳﻴﻨﺜﺎﺱ ﺍﻟﺴﺘﺮﺍﺕ‪ .‬ﻳﺰﻳﺪ ﺍﺭﺗﺒﺎﻁ ‪ OAA‬ﺑﺸﻜﻞ ﻛﺒﻴﺮ ﻣﻦ ﺗﻘﺎﺭﺏ ﺍﻹﻧﺰﻳﻢ ﻣﻊ‬
‫‪] .acetyl CoA‬ﻣﻼﺣﻈﺔ‪ :‬ﺍﻟﺴﻴﺘﺮﺍﺕ ‪ ،‬ﺑﺎﻹﺿﺎﻓﺔ ﺇﻟﻰ ﻛﻮﻧﻬﺎ ﻭﺳﻴﻄﺔ ﻓﻲ ﺩﻭﺭﺓ ‪ ، TCA‬ﻫﻲ ﺃ‬
 ‫ﻣﺼﺪﺭﺍﻷﺳﻴﺘﻴﻞ ‪ CoA‬ﻟﻠﺘﺨﻠﻴﻖ ﺍﻟﻌﺼﺎﺭﻱ ﺍﻟﺨﻠﻮﻱ ﻟﻸﺣﻤﺎﺽ ﺍﻟﺪﻫﻨﻴﺔ )ﺍﻧﻈﺮ ﺹ ‪(183‬‬
‫ﻭﺍﻟﻜﻮﻟﻴﺴﺘﺮﻭﻝ)ﺍﻧﻈﺮ ﺹ ‪ .(220‬ﺍﻟﺴﻴﺘﺮﺍﺕ ﻳﻤﻨﻊ ﺃﻳﻀﺎﻓﺴﻔﻮﻓﺮﻛﺘﻮﻛﻴﻨﺎﺯ ‪، (PFK-1) 1-‬‬
‫ﺇﻧﺰﻳﻢﻳﺤﺪ ﻣﻦ ﻣﻌﺪﻝ ﺗﺤﻠﻞ ﺍﻟﺴﻜﺮ )ﺍﻧﻈﺮ ﺹ ‪ ، (99‬ﻭﻳﻨﺸﻂ ﺃﺳﻴﺘﻴﻞ ‪ CoA‬ﻛﺮﺑﻮﻛﺴﻴﻼﺯ)‬
‫ﺇﻧﺰﻳﻢﻳﺤﺪ ﻣﻦ ﻣﻌﺪﻝ ﺗﺼﻨﻴﻊ ﺍﻷﺣﻤﺎﺽ ﺍﻟﺪﻫﻨﻴﺔ ‪ ،‬ﺍﻧﻈﺮ ﺹ ‪[.(183‬‬
 ‫ﺍﻟﺸﻜﻞ‪ 9.4‬ﺗﺸﻜﻴﻞ ﺃﻟﻔﺎ ﻛﻴﺘﻮﺟﻠﻮﺗﺎﺭﺍﺕ ﻣﻦ ﺃﺳﻴﺘﻴﻞ ﺃﻧﺰﻳﻢ ﺃ )‪ (CoA‬ﻭﺃﻭﻛﺴﺎﻟﻮ ﺃﺳﻴﺘﺎﺕ‬
‫‪ = NAD )H( .‬ﻧﻴﻜﻮﺗﻴﻨﺎﻣﻴﺪ ﺍﻷﺩﻳﻨﻴﻦ ﺛﻨﺎﺉﻲ ﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪ ؛ ﻛﻮ‪ =2‬ﻛﺮﺑﻮﻥ‬
‫ﺛﺎﻧﻲﺃﻛﺴﻴﺪ‪.‬‬

‫ﺟﻴﻢﺃﺯﻣﺮﺓ ﺍﻟﺴﻴﺘﺮﺍﺕ‬
‫ﻳﺘﻢﺇﻳﺰﻭﻣﺮﺓ ﺍﻟﺴﻴﺘﺮﺍﺕ ﺇﻟﻰ ‪ isocitrate‬ﻣﻦ ﺧﻼﻝ ﻫﺠﺮﺓ ﻣﺠﻤﻮﻋﺔ ﺍﻟﻬﻴﺪﺭﻭﻛﺴﻴﻞ ﺍﻟﺘﻲ ﻳﺘﻢ‬
‫ﺗﺤﻔﻴﺰﻫﺎﺑﻮﺍﺳﻄﺔﺃﻛﻮﻧﻴﺘﺎﺯ)ﺃﻛﻮﻧﺖ ﻫﻴﺪﺭﺍﺗﺎﺯ( ‪ ،‬ﻭﻫﻮ ﺑﺮﻭﺗﻴﻦ ﻣﻦ ﺍﻟﺤﺪﻳﺪ ﻭﺍﻟﻜﺒﺮﻳﺖ )ﺍﻧﻈﺮ‬
‫ﺍﻟﺸﻜﻞ‪] .(9.4‬ﻣﻠﺤﻮﻇﺔ‪:‬ﺃﻛﻮﻧﻴﺘﺎﺯﻳﺘﻢ ﺗﺜﺒﻴﻄﻪ ﺑﻮﺍﺳﻄﺔ ‪ ، fluoroacetate‬ﻭﻫﻮ ﺳﻢ ﻧﺒﺎﺗﻲ‬
‫ﻳﺴﺘﺨﺪﻡﻛﻤﺒﻴﺪ ﻟﻶﻓﺎﺕ‪ .‬ﻳﺘﻢ ﺗﺤﻮﻳﻞ ﻓﻠﻮﺭﻭ ﺃﺳﻴﺘﺎﺕ ﺇﻟﻰ ﻓﻠﻮﺭﻭ ﺃﺳﻴﺘﻴﻞ ‪ CoA‬ﺍﻟﺬﻱ ﻳﺘﻜﺜﻒ‬
‫ﻣﻊ‪ OAA‬ﻟﺘﻜﻮﻳﻦ ﻓﻠﻮﺭﻭﺳﻴﺘﺮﺍﺕ ‪ ،‬ﻭﻫﻮ ﻣﺜﺒﻂ ﻗﻮﻱ ﻟـﺃﻛﻮﻧﻴﺘﺎﺯ‪[.‬‬

‫‪ isocitrate‬ﻧﺰﻉ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ ﺍﻟﻤﺆﻛﺴﺪ ﻣﻦ ‪D.‬‬

‫ﻣﺆﻛﺴﺪ‬ ‫ﻻﺭﺟﻌﺔ ﻓﻴﻪ‬ ‫ﺍﻝ‬ ‫ﻳﺤﻔﺰ‬ ‫ﻧﺎﺯﻋﺔﺍﻟﻬﻴﺪﺭﻭﺟﻴﻦ‬ ‫ﺍﻳﺰﻭﺳﺘﺮﺍﺕ‬
‫ﻧﺰﻉﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ ﻣﻦ ‪ isocitrate‬ﺇﻟﻰ ‪ ، α-ketoglutarate‬ﻣﻤﺎ ﻳﻨﺘﺞ ﻋﻨﻪ ﺃﻭﻝ ﺟﺰﻳﺉﺎﺕ‬
‫‪ NADH‬ﺍﻟﺜﻼﺛﺔ ﺍﻟﺘﻲ ﺗﻨﺘﺠﻬﺎ ﺍﻟﺪﻭﺭﺓ ﻭﺍﻹﻃﻼﻕ ﺍﻷﻭﻝ ﻟﺜﺎﻧﻲ ﺃﻛﺴﻴﺪ ﺍﻟﻜﺮﺑﻮﻥ‪)2‬ﻧﺮﻯ‬
‫ﺍﻟﺸﻜﻞ‪ .(9.4‬ﻫﺬﻩ ﺇﺣﺪﻯ ﺧﻄﻮﺍﺕ ﺗﺤﺪﻳﺪ ﺍﻟﻤﻌﺪﻝ ﻟﺪﻭﺭﺓ ‪ .TCA‬ﺍﻝ‬
‫ﻳﺘﻢﺗﻨﺸﻴﻂ ﺍﻹﻧﺰﻳﻢ ﺑﻮﺍﺳﻄﺔ ‪) ADP‬ﺇﺷﺎﺭﺓ ﻣﻨﺨﻔﻀﺔ ﺍﻟﻄﺎﻗﺔ( ﻭ ‪+2Ca‬‬
‫ﻭﻳﺘﻢﺗﺜﺒﻴﻄﻪ ﺑﻮﺍﺳﻄﺔ ‪ ATP‬ﻭ ‪ ، NADH‬ﺣﻴﺚ ﺗﺮﺗﻔﻊ ﻣﺴﺘﻮﻳﺎﺗﻬﻤﺎ ﻋﻨﺪﻣﺎ ﺗﺤﺘﻮﻱ ﺍﻟﺨﻠﻴﺔ‬
‫ﻋﻠﻰﻣﺨﺎﺯﻥ ﻃﺎﻗﺔ ﻭﻓﻴﺮﺓ‪.‬‬

‫ﻛﻴﺘﻮﺟﻠﻮﺗﺎﺭﺍﺕ‪ α-‬ﻧﺰﻉ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ ﺍﻟﻤﺆﻛﺴﺪ ﻣﻦ ‪E.‬‬

‫ﻳﺘﻢﺗﺤﻔﻴﺰ ﺍﻟﺘﺤﻮﻳﻞ ﺍﻟﻨﻬﺎﺉﻲ ﻟـ ‪ α-ketoglutarate‬ﺇﻟﻰ ‪ succinyl CoA‬ﺑﻮﺍﺳﻄﺔﻣﺮﻛﺐ‬
‫ﻧﺎﺯﻋﺔﺍﻟﻬﻴﺪﺭﻭﺟﻴﻦ ﺃﻟﻔﺎ ﻛﻴﺘﻮﺟﻠﻮﺗﺎﺭﺍﺕ‪ ،‬ﻣﺠﻤﻮﻋﺔ ﺑﺮﻭﺗﻴﻨﻴﺔ ﻣﻦ ﻧﺴﺦ ﻣﺘﻌﺪﺩﺓ ﻣﻦ ﺛﻼﺛﺔ‬
‫ﺇﻧﺰﻳﻤﺎﺕ)ﺍﻟﺸﻜﻞ ‪ .(9.5‬ﺁﻟﻴﺔ ﻧﺰﻉ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ ﺍﻟﻤﺆﻛﺴﺪ ﻫﺬﻩ ﻣﺸﺎﺑﻬﺔ ﺟﺪﺍً ﻟﺘﻠﻚ‬
‫ﺍﻟﻤﺴﺘﺨﺪﻣﺔﻓﻲ ﺗﺤﻮﻳﻞ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ ﺇﻟﻰ ﺃﺳﻴﺘﻴﻞ ‪ CoA‬ﺑﻮﺍﺳﻄﺔ‪ .PDHC‬ﻳﻄﻠﻖ ﺍﻟﺘﻔﺎﻋﻞ‬
‫ﺛﺎﻧﻲﺃﻛﺴﻴﺪ ﺍﻟﻜﺮﺑﻮﻥ ‪2CO‬‬
‫ﻭﺗﻨﺘﺞﺛﺎﻧﻲ ‪ NADH‬ﻣﻦ ﺍﻟﺪﻭﺭﺓ‪ .‬ﺍﻹﻧﺰﻳﻤﺎﺕ ﺍﻟﻤﺴﺎﻋﺪﺓ ﺍﻟﻤﻄﻠﻮﺑﺔ ﻫﻲ‬
‫‪ ، FAD ، NAD‬ﺣﻤﺾ ﻟﻴﺒﻮﻳﻚ ‪ ،+TPP ،‬ﻭ ‪ .CoA‬ﺗﻌﻤﻞ ﻛﻞ ﻭﺍﺣﺪﺓ ﻛﺠﺰء ﻣﻦ ﺍﻵﻟﻴﺔ‬
‫ﺍﻟﺘﺤﻔﻴﺰﻳﺔﺑﻄﺮﻳﻘﺔ ﻣﻤﺎﺛﻠﺔ ﻟﺘﻠﻚ ﺍﻟﻤﻮﺻﻮﻓﺔ ﻟـ‪PDHC‬‬
‫)ﺍﻧﻈﺮ ﺹ ‪ G .(110‬ﺍﻟﺴﻠﺒﻲ ﺍﻟﻜﺒﻴﺮ‪0‬ﻣﻦ ﺍﻟﺘﻔﺎﻋﻞ ﻳﻔﻀﻞ ﺗﻜﻮﻳﻦ ‪ ، succinyl CoA‬ﻭﻫﻮ‬
‫ﺛﻴﻮﻳﺴﺘﺮﻋﺎﻟﻲ ﺍﻟﻄﺎﻗﺔ ﻣﺸﺎﺑﻪ ﻷﺳﻴﺘﻴﻞ ‪ .CoA‬ﺍﻝﻣﺮﻛﺐ ﻧﺎﺯﻋﺔ ﺍﻟﻬﻴﺪﺭﻭﺟﻴﻦ ‪-α‬‬
‫ﻛﻴﺘﻮﺟﻠﻮﺗﺎﺭﺍﺕﺗﻤﻨﻌﻪ ﻣﻨﺘﺠﺎﺗﻪ ‪NADH ،‬‬
‫ﻭ‪ ، succinyl CoA‬ﻭﻳﺘﻢ ﺗﻨﺸﻴﻄﻪ ﺑﻮﺍﺳﻄﺔ ‪ .+2Ca‬ﻭﻣﻊ ﺫﻟﻚ ‪ ،‬ﻻ ﻳﺘﻢ ﺗﻨﻈﻴﻤﻬﺎ ﻣﻦ ﺧﻼﻝ‬
‫ﺗﻔﺎﻋﻼﺕﺍﻟﻔﺴﻔﺮﺓ ‪ /‬ﻧﺰﻉ ﺍﻟﻔﺴﻔﺮﺓ ﻛﻤﺎ ﻫﻮ ﻣﻮﺻﻮﻑ ﻟـ‪] .PDHC‬ﻣﻼﺣﻈﺔ‪ :‬ﻳﺘﻢ ﺇﻧﺘﺎﺝ‬
‫‪ α-Ketoglutarate‬ﺃﻳﻀﺎً ﻋﻦ ﻃﺮﻳﻖ ﻧﺰﻉ ﺍﻷﻣﻴﻦ ﺍﻟﻤﺆﻛﺴﺪ )ﺍﻧﻈﺮ ﺹ ‪ (252‬ﻭﻧﻘﻞ ﺣﻤﺾ‬
‫ﺍﻟﻐﻠﻮﺗﺎﻣﺎﺕﺍﻷﺣﻤﺎﺽ ﺍﻷﻣﻴﻨﻴﺔ )ﺍﻧﻈﺮ ﺹ ‪[.(250‬‬
 ‫ﺍﻟﺸﻜﻞ‪ 9.5‬ﺗﻜﻮﻳﻦ ﻣﺎﻻﺕ ﻣﻦ ‪ (2α-ketoglutarate. FAD )H‬ﻭ (‪= NAD )H‬‬
‫ﺍﻟﻔﻼﻓﻴﻦﻭﺍﻟﻨﻴﻜﻮﺗﻴﻨﺎﻣﻴﺪ ﺍﻷﺩﻳﻨﻴﻦ ﺛﻨﺎﺉﻲ ﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪﺍﺕ ؛ ﺍﻟﻨﺎﺗﺞ ﺍﻟﻤﺤﻠﻲ ﺍﻹﺟﻤﺎﻟﻲ ﻭ ‪ = GTP‬ﻏﻮﺍﻧﻮﺯﻳﻦ‬
‫ﺛﻨﺎﺉﻲﻭﺛﻼﺛﻲ ﺍﻟﻔﻮﺳﻔﺎﺕ ؛ ~ = ﺭﺍﺑﻄﺔ ﻋﺎﻟﻴﺔ ﺍﻟﻄﺎﻗﺔ ؛ ‪ = CoA‬ﺃﻧﺰﻳﻢ ﺃ‪.‬‬

‫ﺍﻻﻧﻘﺴﺎﻡ‪ Succinyl‬ﺍﻧﺰﻳﻢ ‪F.‬‬

‫ﺳﻜﺴﻴﻨﺎﺕﺛﻴﻮﻛﻴﻨﺎﺯ)ﺃﻳﻀﺎ ﻳﺴﻤﻰﺇﻧﺰﻳﻢ ﺍﻟﺴﻜﺴﻴﻨﻴﻞ ‪ ،CoA synthetase‬ﺍﻟﻤﺴﻤﻰ‬
‫ﻟﻠﺘﻔﺎﻋﻞﺍﻟﻌﻜﺴﻲ( ﻳﺸﻖ ﺭﺍﺑﻄﺔ ‪ thioester‬ﻋﺎﻟﻴﺔ ﺍﻟﻄﺎﻗﺔ ﻣﻦ ‪) succinyl CoA‬ﺍﻧﻈﺮ‬
‫ﺍﻟﺸﻜﻞ‪ .(9.5‬ﻳﻘﺘﺮﻥ ﻫﺬﺍ ﺍﻟﺘﻔﺎﻋﻞ ﺑﻔﺴﻔﺮﺓ ﻏﻮﺍﻧﻮﺯﻳﻦ ﺛﻨﺎﺉﻲ ﻓﻮﺳﻔﺎﺕ )ﺍﻟﻨﺎﺗﺞ ﺍﻟﻤﺤﻠﻲ‬
‫ﺍﻹﺟﻤﺎﻟﻲ( ﺇﻟﻰ ﻏﻮﺍﻧﻮﺯﻳﻦ ﺛﻼﺛﻲ ﺍﻟﻔﻮﺳﻔﺎﺕ )‪ GTP .(GTP‬ﻭ ‪ ATP‬ﻗﺎﺑﻠﺔ ﻟﻠﺘﺤﻮﻳﻞ ﺍﻟﺒﻴﻨﻲ‬
‫ﺑﻘﻮﺓﺑﻮﺍﺳﻄﺔﻧﻴﻮﻛﻠﻴﻮﺯﻳﺪ ﺛﻨﺎﺉﻲ ﻓﻮﺳﻔﺎﺕ ﻛﻴﻨﺎﺯ ﺗﻔﺎﻋﻞ‪:‬‬

‫ﺟﻴﻞ‪ GTP‬ﺑﻮﺍﺳﻄﺔﺳﻜﺴﻴﻨﺎﺕ ﺛﻴﻮﻛﻴﻨﺎﺯﻣﺜﺎﻝ ﺁﺧﺮ ﻋﻠﻰ ﺍﻟﻔﺴﻔﺮﺓ ﻋﻠﻰ ﻣﺴﺘﻮﻯ ﺍﻟﺮﻛﻴﺰﺓ )‬

‫ﺍﻧﻈﺮﺹ ‪] .(102‬ﻣﻼﺣﻈﺔ‪ :‬ﻳﺘﻢ ﺇﻧﺘﺎﺝ ‪ Succinyl CoA‬ﺃﻳﻀﺎً ﻣﻦ ﺑﺮﻭﺑﻴﻮﻧﻴﻞ ‪ CoA‬ﺍﻟﻤﺸﺘﻖ‬
‫ﻣﻦﻋﻤﻠﻴﺔ ﺍﻟﺘﻤﺜﻴﻞ ﺍﻟﻐﺬﺍﺉﻲ ﻟﻸﺣﻤﺎﺽ ﺍﻟﺪﻫﻨﻴﺔ ﻣﻊ ﻋﺪﺩ ﻓﺮﺩﻱ ﻣﻦ ﺫﺭﺍﺕ ﺍﻟﻜﺮﺑﻮﻥ )ﺍﻧﻈﺮ‬
‫ﺍﻟﺼﻔﺤﺔ‪ (193‬ﻭﻣﻦ ﻋﻤﻠﻴﺔ ﺍﻟﺘﻤﺜﻴﻞ ﺍﻟﻐﺬﺍﺉﻲ ﻟﻠﻌﺪﻳﺪ ﻣﻦ ﺍﻷﺣﻤﺎﺽ ﺍﻷﻣﻴﻨﻴﺔ )ﺍﻧﻈﺮ‬
‫ﺍﻟﺼﻔﺤﺎﺕ‪ .(266-265‬ﻳﻤﻜﻦ ﺗﺤﻮﻳﻠﻪ ﺇﻟﻰ ﺑﻴﺮﻭﻓﺎﺕ ﻟﺘﻜﻮﻳﻦ ﺍﻟﺴﻜﺮ )ﺍﻧﻈﺮ ﺹ ‪ (118‬ﺃﻭ‬
‫ﺍﺳﺘﺨﺪﺍﻣﻪﻓﻲ ﺗﺨﻠﻴﻖ ﺍﻟﻬﻴﻢ )ﺍﻧﻈﺮ ﺹ ‪[.(278‬‬

‫ﺃﻛﺴﺪﺓﺳﻜﺴﻴﻨﺎﺕ‬
‫ﻳﺘﺄﻛﺴﺪﺳﻜﺴﻴﻨﺎﺕ ﺇﻟﻰ ﻓﻮﻣﺎﺭﺍﺕ ﺑﻮﺍﺳﻄﺔﻧﺎﺯﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻦ ﺍﻟﺴﻜﺴﻴﻨﺎﺕ‪ ،‬ﺣﻴﺚ ﻳﺘﻢ‬
‫ﺗﻘﻠﻴﻞﺍﻹﻧﺰﻳﻢ ﺍﻟﻤﺴﺎﻋﺪ ‪ FAD‬ﺇﻟﻰ ‪)2FADH‬ﻧﺮﻯﺍﻟﺸﻜﻞ ‪.(9.5‬ﺳﻜﺴﻴﻨﺎﺕ‬
‫ﻧﺎﺯﻋﺔﺍﻟﻬﻴﺪﺭﻭﺟﻴﻦﻫﻮ ﺍﻹﻧﺰﻳﻢ ﺍﻟﻮﺣﻴﺪ ﻟﺪﻭﺭﺓ ‪ TCA‬ﺍﻟﻤﺪﻣﺞ ﻓﻲ ﻏﺸﺎء ﺍﻟﻤﻴﺘﻮﻛﻮﻧﺪﺭﻳﺎ ﺍﻟﺪﺍﺧﻠﻲ‪.‬‬
‫ﻋﻠﻰﻫﺬﺍ ﺍﻟﻨﺤﻮ ‪ ،‬ﻓﺈﻧﻪ ﻳﻌﻤﻞ ﻛﻤﺠﻤﻊ ‪ II‬ﻣﻦ‬
‫‪ NAD‬ﺑﺪﻻ ًﻣﻦ ‪: FAD ،‬ﻣﻼﺣﻈﺔ] ‪(.‬ﺍﻧﻈﺮ ﺹ ‪ ،+ETC )75‬ﻫﻮ ﻣﺘﻘﺒﻞ ﺍﻹﻟﻜﺘﺮﻭﻥ ﻷﻥ ﺍﻟﻘﻮﺓ‬
‫ﺍﻟﻤﺨﺘﺰﻟﺔﻟﻠﺴﻜﺴﻴﻨﺎﺕ ﻟﻴﺴﺖ ﻛﺎﻓﻴﺔ ﻟﺘﻘﻠﻴﻞ ‪[.+NAD‬‬

‫ﺗﺮﻃﻴﺐﻓﻮﻣﺎﺭﺍﺕ ‪H.‬‬
‫ﻳﺘﻢﺗﺮﻃﻴﺐ ﻓﻮﻣﺎﺭﺍﺕ ﺇﻟﻰ ﻣﺎﻻﺕ ﻓﻲ ﺗﻔﺎﻋﻞ ﻗﺎﺑﻞ ﻟﻠﻌﻜﺲ ﻳﺘﻢ ﺗﺤﻔﻴﺰﻩ ﺑﻮﺍﺳﻄﺔ ﻓﻮﻣﺎﺭﺍﺱ)‬
‫ﻓﻮﻣﺎﺭﺍﺕﻫﻴﺪﺭﺍﺗﺎﺯ‪ ،‬ﻧﺮﻯﺍﻟﺸﻜﻞ ‪] .(9.5‬ﻣﻼﺣﻈﺔ‪ :‬ﻓﻮﻣﺎﺭﺍﺕ ﺃﻳﻀﺎ‬
‫ﺍﻟﺘﻲﺗﻨﺘﺠﻬﺎ ﺩﻭﺭﺓ ﺍﻟﻴﻮﺭﻳﺎ )ﺍﻧﻈﺮ ﺹ ‪ ، (255‬ﻓﻲ ﺗﺨﻠﻴﻖ ﺍﻟﺒﻴﻮﺭﻳﻦ )ﺍﻧﻈﺮ ﺍﻟﺸﻜﻞ ‪ 22.7‬ﻓﻲ‬
‫ﺹ‪ ، (294‬ﻭﺃﺛﻨﺎء ﻫﺪﻡ ﺍﻷﺣﻤﺎﺽ ﺍﻷﻣﻴﻨﻴﺔ ﻓﻴﻨﻴﻞ ﺃﻻﻧﻴﻦ ﻭﺗﻴﺮﻭﺯﻳﻦ )ﺍﻧﻈﺮ ﺹ ‪[.(263‬‬

‫ﺃﻛﺴﺪﺓﻣﺎﻻﺕ ‪I.‬‬
‫ﻳﺘﺄﻛﺴﺪ‪ Malate‬ﺇﻟﻰ ‪ OAA‬ﺑﻮﺍﺳﻄﺔﻧﺎﺯﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻦ ﻣﺎﻻﺕ)ﺍﻟﺸﻜﻞ ‪ .(9.6‬ﻫﺬﻩ‬
‫ﻳﻨﺘﺞﺭﺩ ﺍﻟﻔﻌﻞ ‪ NADH‬ﺍﻟﺜﺎﻟﺚ ﻭﺍﻷﺧﻴﺮ ﻣﻦ ﺍﻟﺪﻭﺭﺓ‪0G∆ .‬ﻣﻦ ﺭﺩ ﺍﻟﻔﻌﻞ ﺇﻳﺠﺎﺑﻲ ‪ ،‬ﻭﻟﻜﻦ ﺭﺩ‬
‫ﺍﻟﻔﻌﻞﻣﺪﻓﻮﻉ ﻓﻲ ﺍﺗﺠﺎﻩ ‪ OAA‬ﺑﻮﺍﺳﻄﺔ ﻃﺎﻗﺔ ﻋﺎﻟﻴﺔﺳﻴﻨﺜﺎﺱ ﺍﻟﺴﺘﺮﺍﺕﺗﻔﺎﻋﻞ‪] .‬ﻣﻼﺣﻈﺔ‪:‬‬
‫ﻳﺘﻢﺇﻧﺘﺎﺝ ﺍﻟﺰﺭﺍﻋﺔ ﺍﻟﻌﻀﻮﻳﺔ ‪ A‬ﺃﻳﻀﺎً ﻋﻦ ﻃﺮﻳﻖ ﻧﻘﻞ ﺣﻤﺾ ﺍﻷﺳﺒﺎﺭﺗﻴﻚ ﻣﻦ ﺍﻷﺣﻤﺎﺽ‬
‫ﺍﻷﻣﻴﻨﻴﺔ)ﺍﻧﻈﺮ ﺍﻟﺼﻔﺤﺔ ‪[.(250‬‬
‫ﺍﻟﺸﻜﻞ‪ 9.6‬ﺗﻜﻮﻳﻦ )ﺗﺠﺪﻳﺪ( ﺃﻭﻛﺴﺎﻟﻮ ﺃﺳﻴﺘﺎﺕ ﻣﻦ ﻣﺎﻻﺕ‪ = NAD )H( .‬ﻧﻴﻜﻮﺗﻴﻨﺎﻣﻴﺪ ﺍﻷﺩﻳﻨﻴﻦ‬
‫ﺛﻨﺎﺉﻲﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪ‪.‬‬

‫ﺛﺎﻟﺜﺎ‪.‬ﺍﻟﻄﺎﻗﺔ ﺍﻟﺘﻲ ﺗﻨﺘﺠﻬﺎ ﺍﻟﺪﻭﺭﺓ‬

‫ﻳﺘﻢﻧﻘﻞ ﺃﺭﺑﻌﺔ ﺃﺯﻭﺍﺝ ﻣﻦ ﺍﻹﻟﻜﺘﺮﻭﻧﺎﺕ ﺧﻼﻝ ﺩﻭﺭﺓ ﻭﺍﺣﺪﺓ ﻣﻦ ﺩﻭﺭﺓ ‪ :TCA‬ﺛﻼﺛﺔ ﺃﺯﻭﺍﺝ ﺗﻘﻠﻞ ﺛﻼﺛﺔ‬
‫‪+NAD‬ﺇﻟﻰ ‪ NADH‬ﻭﺯﻭﺝ ﻭﺍﺣﺪ ﻳﻘﻠﻞ ﻣﻦ ‪ FAD‬ﺇﻟﻰ ‪.2FADH‬‬
‫ﺗﺆﺩﻱﺃﻛﺴﺪﺓ ‪ NADH‬ﺑﻮﺍﺳﻄﺔ ‪ ETC‬ﺇﻟﻰ ﺗﻜﻮﻳﻦ ﺛﻼﺛﺔ ‪ ، ATP‬ﻓﻲ ﺣﻴﻦ ﺃﻥ ﺃﻛﺴﺪﺓ ‪2FADH‬ﻳﻨﺘﺞ‬
‫ﺍﺛﻨﻴﻦﻣﻦ ‪) ATP‬ﺍﻧﻈﺮ ﺹ ‪ .(77‬ﺇﺟﻤﺎﻟﻲ ﺍﻟﻌﺎﺉﺪ ﻣﻦ ‪ ATP‬ﻣﻦ‬
 ‫ﻳﻈﻬﺮﺃﻛﺴﺪﺓ ﺃﺣﺪ ﺍﻷﺳﻴﺘﻴﻞ ‪ CoA‬ﻓﻲﺍﻟﺸﻜﻞ ‪.9.7‬ﺍﻟﺸﻜﻞ ‪9.8‬ﻳﻠﺨﺺ ﺗﻔﺎﻋﻼﺕ ﺩﻭﺭﺓ ‪] .TCA‬‬
‫ﻣﻼﺣﻈﺔ‪:‬ﻻ ﺗﺘﻀﻤﻦ ﺍﻟﺪﻭﺭﺓ ﺻﺎﻓﻲ ﺍﺳﺘﻬﻼﻙ ﺃﻭ ﺇﻧﺘﺎﺝ ﺍﻟﻤﻮﺍﺩ ﺍﻟﻮﺳﻴﻄﺔ‪ .‬ﻳﺘﻢ ﻣﻮﺍﺯﻧﺔ ﺍﺛﻨﻴﻦ ﻣﻦ‬
‫ﺍﻟﻜﺮﺑﻮﻥﺍﻟﻠﺬﺍﻥ ﻳﺪﺧﻼﻥ ﻛﺄﺳﻴﺘﻴﻞ ‪ CoA‬ﺑﻮﺍﺳﻄﺔ ﺍﺛﻨﻴﻦ ﻣﻦ ﺛﺎﻧﻲ ﺃﻛﺴﻴﺪ ﺍﻟﻜﺮﺑﻮﻥ‪2‬ﺍﻟﺨﺮﻭﺝ‪[.‬‬

‫ﺍﻟﺸﻜﻞ‪ 9.7‬ﻋﺪﺩ ﺟﺰﻳﺉﺎﺕ ‪ ATP‬ﺍﻟﻨﺎﺗﺠﺔ ﻋﻦ ﺃﻛﺴﺪﺓ ﺟﺰﻱء ﻭﺍﺣﺪ ﻣﻦ ﺃﻧﺰﻳﻢ ﺍﻷﺳﻴﺘﻴﻞ ﺍﻟﻤﺴﺎﻋﺪ‬
‫(‪ A )CoA‬ﺑﺎﺳﺘﺨﺪﺍﻡ ﺍﻟﻔﺴﻔﺮﺓ ﻋﻠﻰ ﻣﺴﺘﻮﻯ ﺍﻟﺮﻛﻴﺰﺓ ﻭﺍﻟﺘﺄﻛﺴﺪ‪ NAD )H( .‬ﻭ ‪= (2FAD )H‬‬
‫ﻧﻴﻜﻮﺗﻴﻨﺎﻣﻴﺪﻭﻓﻼﻓﻴﻦ ﺃﺩﻳﻨﻴﻦ‬
‫ﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪﺍﺕ‪.‬ﺍﻟﻨﺎﺗﺞ ﺍﻟﻤﺤﻠﻲ ﺍﻹﺟﻤﺎﻟﻲ ﻭ ‪ = GTP‬ﻏﻮﺍﻧﻮﺯﻳﻦ ﺛﻨﺎﺉﻲ ﻭﺛﻼﺛﻲ ﺍﻟﻔﻮﺳﻔﺎﺕ ؛ ﺹﺃﻧﺎ= ﻓﻮﺳﻔﺎﺕ ﻏﻴﺮ‬
‫ﻋﻀﻮﻱ‪.‬‬
 ‫ﺍﻟﺸﻜﻞ‪ 9.8‬ﺃ‪.‬ﺇﻧﺘﺎﺝ ﺍﻹﻧﺰﻳﻤﺎﺕ ﺍﻟﻤﺨﺘﺰﻟﺔ ‪ ، ATP ،‬ﻭﺛﺎﻧﻲ ﺃﻛﺴﻴﺪ ﺍﻟﻜﺮﺑﻮﻥ )‪(2CO‬‬
‫ﻓﻲﺩﻭﺭﺓ ﺣﻤﺾ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ‪] .‬ﻣﻼﺣﻈﺔ‪ :‬ﻳﺘﻢ ﺗﺤﻮﻳﻞ ﻏﻮﺍﻧﻮﺯﻳﻦ ﺛﻼﺛﻲ ﺍﻟﻔﻮﺳﻔﺎﺕ )‪ (GTP‬ﻭ ‪ATP‬‬
‫ﺑﻮﺍﺳﻄﺔﻧﻴﻮﻛﻠﻴﻮﺯﻳﺪ ﺛﻨﺎﺉﻲ ﻓﻮﺳﻔﺎﺕ ﻛﻴﻨﺎﺯ‪ [.‬ﺏ‪ .‬ﻣﺜﺒﻄﺎﺕ ﻭﻣﻨﺸﻄﺎﺕ ﺍﻟﺪﻭﺭﺓ‪.‬‬

‫ﺭﺍﺑﻌﺎ‪.‬ﺗﻨﻈﻴﻢ ﺍﻟﺪﻭﺭﺓ‬
‫ﻋﻠﻰﻋﻜﺲ ﺗﺤﻠﻞ ﺍﻟﺴﻜﺮ ‪ ،‬ﺍﻟﺬﻱ ﻳﻨﻈﻤﻪ ﻓﻲ ﺍﻟﻤﻘﺎﻡ ﺍﻷﻭﻝ‪ ،PFK-1‬ﻳﺘﻢ ﺍﻟﺘﺤﻜﻢ ﻓﻲ ﺩﻭﺭﺓ ‪ TCA‬ﻣﻦ‬
‫ﺧﻼﻝﺗﻨﻈﻴﻢ ﺍﻟﻌﺪﻳﺪ ﻣﻦ ﺍﻹﻧﺰﻳﻤﺎﺕ )ﺍﻧﻈﺮﺍﻟﺸﻜﻞ ‪ .(9.8‬ﺃﻫﻢ ﻫﺬﻩ ﺍﻹﻧﺰﻳﻤﺎﺕ ﺍﻟﻤﻨﻈﻤﺔ ﻫﻲ ﺗﻠﻚ‬
‫ﺍﻟﺘﻲﺗﺤﻔﺰ ﺍﻟﺘﻔﺎﻋﻼﺕ‬
‫ﺳﻠﺒﻲﻟﻠﻐﺎﻳﺔ ‪:0∆G‬ﺳﻴﻨﺜﺎﺱ ﺍﻟﺴﺘﺮﺍﺕﻭﻧﺎﺯﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻦ ﺍﻷﻳﺰﻭﺳﺘﺮﺍﺕ‪ ،‬ﻭ ﺍﻝﻣﺮﻛﺐ ﻧﺎﺯﻋﺔ‬
‫ﺍﻟﻬﻴﺪﺭﻭﺟﻴﻦ‪ -α‬ﻛﻴﺘﻮﺟﻠﻮﺗﺎﺭﺍﺕ‪ .‬ﻳﺘﻢ ﺇﻧﺸﺎء ﺗﻘﻠﻴﻞ ﺍﻟﻤﻌﺎﺩﻻﺕ ﺍﻟﻼﺯﻣﺔ ﻟﻠﻔﺴﻔﺮﺓ ﺍﻟﻤﺆﻛﺴﺪﺓ‬
‫ﺑﻮﺍﺳﻄﺔ‪PDHC‬ﻭﺩﻭﺭﺓ ‪ ، TCA‬ﻭﻛﻼ ﺍﻟﻌﻤﻠﻴﺘﻴﻦ ﻣﻨﻈﻤﺘﺎﻥ ﺍﺳﺘﺠﺎﺑﺔ ﻻﻧﺨﻔﺎﺽ ﻧﺴﺒﺔ ‪.ATP / ADP‬‬
 ‫ﺧﺎﻣﺴﺎ‪ -‬ﻣﻠﺨﺺ ﺍﻟﻔﺼﻞ‬
‫ﺑﻴﺮﻭﻓﺎﺕﻣﻨﺰﻭﻋﺔ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ ﻣﺆﻛﺴﺪ ﺑﻮﺍﺳﻄﺔﻣﺮﻛﺐ ﻧﺎﺯﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻦ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ)‬
‫‪ ، (PDHC‬ﻭﺇﻧﺘﺎﺝ ﺃﻧﺰﻳﻢ ﺃﺳﻴﺘﻴﻞ (‪ ، A )CoA‬ﻭﻫﻮ ﺍﻟﻮﻗﻮﺩ ﺍﻟﺮﺉﻴﺴﻲ ﻟﺪﻭﺭﺓ ﺣﻤﺾ‬
‫ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻠﻴﻚ)‪) (TCA‬ﺍﻟﺸﻜﻞ ‪ .(9.9‬ﻣﺘﻌﺪﺩ ﺍﻹﻧﺰﻳﻢ ‪PDHC‬ﻳﺘﻄﻠﺐ ﺧﻤﺴﺔ ﺃﻧﺰﻳﻤﺎﺕ‬
‫ﻣﺴﺎﻋﺪﺓ‪:‬ﺛﻴﺎﻣﻴﻦ ﺑﻴﺮﻭﻓﻮﺳﻔﺎﺕ ‪ ،‬ﺣﻤﺾ ﻟﻴﺒﻮﻳﻚ ‪ ،‬ﻓﻼﻓﻴﻦ ﺃﺩﻳﻨﻴﻦ ﺛﻨﺎﺉﻲ ﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪ‬
‫)‪ ، (FAD‬ﻧﻴﻜﻮﺗﻴﻨﺎﻣﻴﺪ ﺃﺩﻳﻨﻴﻦ ﺛﻨﺎﺉﻲ ﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪ‬
‫‪ (+)NAD‬ﻭ ‪ .CoA‬ﺍﻝ‪PDHC‬ﻳﻨﻈﻢ ﺍﻟﺘﻌﺪﻳﻞ ﺍﻟﺘﺴﺎﻫﻤﻲ ﻟـﻩ ‪) 1‬ﺑﻴﺮﻭﻓﺎﺕ ﺩﻳﻜﺎﺭﺑﻮﻛﺴﻴﻼﺯ(‬
‫ﺑﻮﺍﺳﻄﺔﻛﻴﻨﺎﺯ ‪PDH‬ﻭﺇﻧﺰﻳﻢ ‪ PDH‬ﺍﻟﻔﻮﺳﻔﺎﺗﻴﺰ‪ :‬ﻣﺜﺒﻄﺎﺕ ﺍﻟﻔﺴﻔﺮﺓﻩ ‪.1‬ﻛﻴﻨﺎﺯ ‪PDH‬ﻳﺘﻢ‬
‫ﺗﻨﺸﻴﻄﻪﺑﻮﺍﺳﻄﺔ ‪ ATP‬ﻭ ‪ acetyl CoA‬ﻭ ‪ NADH‬ﻭﻳﻤﻨﻌﻪ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ‪ .‬ﺍﻝﺍﻟﻔﻮﺳﻔﺎﺗﻴﺰ‬

‫ﺑﻮﺍﺳﻄﺔﺍﻟﻜﺎﻟﺴﻴﻮﻡ )‪.(+2Ca‬ﻩ ‪1‬ﺍﻟﻨﻘﺺ ﻫﻮ ﺍﻟﺴﺒﺐ ﺍﻟﺒﻴﻮﻛﻴﻤﻴﺎﺉﻲ ﺍﻷﻛﺜﺮ ﺷﻴﻮﻋﺎً ﻟﻠﺤﻤﺎﺽ‬

‫ﺍﻟﻠﺒﻨﻲﺍﻟﺨﻠﻘﻲ‪ .‬ﻳﺘﺄﺛﺮ ﺍﻟﺪﻣﺎﻍ ﺑﺸﻜﻞ ﺧﺎﺹ ﻓﻲ ﻫﺬﺍ ﺍﻻﺿﻄﺮﺍﺏ ﺍﻟﺴﺎﺉﺪ ﺍﻟﻤﺮﺗﺒﻂ‬
‫ﺑﺎﻟﻜﺮﻭﻣﻮﺳﻮﻡ‪ .X‬ﻳﺴﺒﺐ ﺍﻟﺘﺴﻤﻢ ﺑﺎﻟﺰﺭﻧﻴﺦ ﺗﻌﻄﻴﻞ‪PDHC‬ﻋﻦ ﻃﺮﻳﻖ ﺍﻻﺭﺗﺒﺎﻁ ﺑﺤﻤﺾ‬
‫ﻟﻴﺒﻮﻳﻚ‪.‬ﻓﻲ ﺩﻭﺭﺓ ‪ ، TCA‬ﻳﺘﻢ ﺗﺼﻨﻴﻊ ﺍﻟﺴﺘﺮﺍﺕ ﻣﻦ (‪ oxaloacetate )OAA‬ﻭ ‪CoA‬‬
‫‪ acetyl‬ﺑﻮﺍﺳﻄﺔﺳﻴﻨﺜﺎﺱ ﺍﻟﺴﺘﺮﺍﺕ‪ ،‬ﺍﻟﺬﻱ ﻳﻤﻨﻌﻪ ﺍﻟﻤﻨﺘﺞ‪ .‬ﻳﺘﻢ ﺇﻳﺰﻭﻣﺮﺓ ﺍﻟﺴﻴﺘﺮﺍﺕ ﺇﻟﻰ‬
‫‪ isocitrate‬ﺑﻮﺍﺳﻄﺔﺃﻛﻮﻧﻴﺘﺎﺯ)ﺃﻛﻮﻧﺖ ﻫﻴﺪﺭﺍﺗﺎﺯﻣﻨﺰﻭﻉ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ ﻣﺆﻛﺴﺪ ﺑﻮﺍﺳﻄﺔ‬
‫‪(. Isocitrate‬ﻧﺎﺯﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻦ ﺍﻷﻳﺰﻭﺳﺘﺮﺍﺕﺇﻟﻰ ‪ ، α-ketoglutarate‬ﻹﻧﺘﺎﺝ ﺛﺎﻧﻲ‬
‫ﺃﻛﺴﻴﺪﺍﻟﻜﺮﺑﻮﻥ )‪ (2CO‬ﻭ ‪ .NADH‬ﻳﺘﻢ ﺗﺜﺒﻴﻂ ﺍﻹﻧﺰﻳﻢ ﺑﻮﺍﺳﻄﺔ ‪ ATP‬ﻭ ‪ NADH‬ﻭ‬

‫ﻳﺘﻢﺗﻨﺸﻴﻄﻪ ﺑﻮﺍﺳﻄﺔ ﺛﻨﺎﺉﻲ ﻓﻮﺳﻔﺎﺕ ﺍﻷﺩﻳﻨﻮﺯﻳﻦ )‪ (ADP‬ﻭ ‪+2Ca‬ﺑﻮﺍﺳﻄﺔ ‪CoA‬‬

‫‪ Succinyl‬ﻣﻨﺰﻭﻉ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ ﻣﺆﻛﺴﺪ ﺇﻟﻰ ‪. α-Ketoglutarate‬ﻣﺮﻛﺐ ﻧﺎﺯﻋﺔ‬
‫ﺍﻟﻬﻴﺪﺭﻭﺟﻴﻦﺃﻟﻔﺎ ﻛﻴﺘﻮﺟﻠﻮﺗﺎﺭﺍﺕ‪ ،‬ﺇﻧﺘﺎﺝ ﺛﺎﻧﻲ ﺃﻛﺴﻴﺪ ﺍﻟﻜﺮﺑﻮﻥ‪2‬ﻭ ‪ .NADH‬ﺍﻻﻧﺰﻳﻢ ﺟﺪﺍ‬
‫ﻣﺸﺎﺑﻬﻪﻝ‪PDHC‬ﻭﻳﺴﺘﺨﺪﻡ ﻧﻔﺲ ﺍﻹﻧﺰﻳﻤﺎﺕ‪ .‬ﺍﻝﺃﻟﻔﺎ ﻛﻴﺘﻮﺟﻠﻮﺗﺎﺭﺍﺕ‬
‫ﻣﺠﻤﻊﻧﺎﺯﻋﺔ ﺍﻟﻬﻴﺪﺭﻭﺟﻴﻦﺑﻮﺍﺳﻄﺔ ‪+2Ca‬ﻭﺗﻢ ﺗﺜﺒﻴﻄﻪ ﺑﻮﺍﺳﻄﺔ ‪ NADH‬ﻭ ‪succinyl CoA‬‬
‫ﻭﻟﻜﻦﻻ ﻳﺘﻢ ﺗﻨﻈﻴﻤﻪ ﺗﺴﺎﻫﻤﻴﺎً‪ .‬ﻣﺸﻘﻮﻕ ‪ Succinyl CoA‬ﺑﻮﺍﺳﻄﺔ ﺳﻜﺴﻴﻨﺎﺕ ﺛﻴﻮﻛﻴﻨﺎﺯ)‬
‫ﺃﻳﻀﺎﻳﺴﻤﻰﺇﻧﺰﻳﻢ ﺍﻟﺴﻜﺴﻴﻨﻴﻞ ‪ ، (CoA synthetase‬ﻭﺇﻧﺘﺎﺝ ﺍﻟﺴﻜﺴﻴﻨﺎﺕ ﻭﻏﻮﺍﻧﻮﺯﻳﻦ‬
‫ﺛﻼﺛﻲﺍﻟﻔﻮﺳﻔﺎﺕ )‪ .(GTP‬ﻫﺬﺍ ﻣﺜﺎﻝ ﻋﻠﻰ ﺍﻟﻔﺴﻔﺮﺓ ﻋﻠﻰ ﻣﺴﺘﻮﻯ ﺍﻟﺮﻛﻴﺰﺓ‪ .‬ﻳﺘﺄﻛﺴﺪ‬
‫ﺳﻜﺴﻴﻨﺎﺕﺇﻟﻰ ﻓﻮﻣﺎﺭﺍﺕ ﺑﻮﺍﺳﻄﺔ ﻧﺎﺯﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻦ ﺍﻟﺴﻜﺴﻴﻨﺎﺕ‪ ،‬ﺇﻧﺘﺎﺝ ‪ .2FADH‬ﻳﺘﻢ‬
‫ﺗﺮﻃﻴﺐﻓﻮﻣﺎﺭﺍﺕ ﻝ‬
‫ﻣﺎﻻﺕﻓﻮﻣﺎﺭﺍﺱ)ﻓﻮﻣﺎﺭﺍﺕ ﻫﻴﺪﺭﺍﺗﺎﺯ( ‪ ،‬ﻭﻳﺘﺄﻛﺴﺪ ‪ malate‬ﺇﻟﻰ ‪ OAA‬ﺑﻮﺍﺳﻄﺔﻧﺎﺯﻋﺔ‬
‫ﻫﻴﺪﺭﻭﺟﻴﻦﻣﺎﻻﺕ‪ ،‬ﻭﺇﻧﺘﺎﺝ ‪ .NADH‬ﺛﻼﺛﺔ ‪ NADH‬ﻭﻭﺍﺣﺪ ‪2FADH‬ﻳﺘﻢ ﺇﻧﺘﺎﺟﻬﺎ ﺑﺠﻮﻟﺔ‬
‫ﻭﺍﺣﺪﺓﻣﻦ ﺩﻭﺭﺓ ‪ .TCA‬ﺟﻴﻞ‬
‫ﺃﺳﻴﺘﻴﻞ‪ CoA‬ﻋﻦ ﻃﺮﻳﻖ ﺃﻛﺴﺪﺓ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ ﻋﺒﺮ‪PDHC‬ﻳﻨﺘﺞ ﺃﻳﻀﺎً ﻣﻠﻒ‬
 ‫‪ FADH‬ﻭ ‪ NADH‬ﺃﻛﺴﺪﺓ ‪2NADH.‬ﺑﻮﺍﺳﻄﺔ ‪ ETC‬ﻳﻨﺘﺞ ‪.ATP 14‬‬
‫ﻳﻤﻜﻦﻧﻘﻞ ﺍﻟﻔﻮﺳﻔﺎﺕ ﺍﻟﻨﻬﺎﺉﻲ ﻟـ ‪ GTP‬ﺍﻟﻨﺎﺗﺞ ﻋﻦ ﺍﻟﻔﺴﻔﺮﺓ ﻋﻠﻰ ﻣﺴﺘﻮﻯ ﺍﻟﺮﻛﻴﺰﺓ ﻓﻲ‬
‫ﺩﻭﺭﺓ‪ TCA‬ﺇﻟﻰ ‪ ADP‬ﺑﻮﺍﺳﻄﺔﻧﻴﻮﻛﻠﻴﻮﺯﻳﺪ ﺛﻨﺎﺉﻲ ﻓﻮﺳﻔﺎﺕ ﻛﻴﻨﺎﺯ‪ ،‬ﻣﻤﺎ ﻳﻨﺘﺞ ﻋﻨﻪ ‪ ATP‬ﺁﺧﺮ‪.‬‬
‫ﻟﺬﻟﻚ ‪،‬ﻳﺘﻢ ﺇﻧﺘﺎﺝ ﻣﺎ ﻣﺠﻤﻮﻋﻪ ‪ ATP 15‬ﻣﻦ ﺃﻛﺴﺪﺓ ﺍﻟﻤﻴﺘﻮﻛﻮﻧﺪﺭﻳﺎ ﺍﻟﻜﺎﻣﻠﺔ ﻣﻦ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ‬
‫ﺇﻟﻰ‪.2CO‬‬
 ‫ﺍﻟﺸﻜﻞ‪ 9.9‬ﺧﺮﻳﻄﺔ ﺍﻟﻤﻔﺎﻫﻴﻢ ﺍﻟﺮﺉﻴﺴﻴﺔ ﻟﺪﻭﺭﺓ ﺣﻤﺾ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ )‪.(TCA‬ﻣﺮﻛﺐ ﻧﺎﺯﻋﺔ‬
‫ﻫﻴﺪﺭﻭﺟﻴﻦﺍﻟﺒﻴﺮﻭﻓﺎﺕ = ‪PDHC‬؛ ‪ = CoA‬ﺃﻧﺰﻳﻢ ‪ A‬؛ ﻛﻮ‪ =2‬ﺛﺎﻧﻲ ﺃﻛﺴﻴﺪ ﺍﻟﻜﺮﺑﻮﻥ‬
‫‪ FAD )H‬ﻧﻴﻜﻮﺗﻴﻨﺎﻣﻴﺪ ﺍﻷﺩﻳﻨﻴﻦ ﺛﻨﺎﺉﻲ ﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪ ؛ = (‪ = (2NAD )H‬ﻓﻼﻓﻴﻦ ﺍﻷﺩﻳﻨﻴﻦ‬
‫ﺛﻨﺎﺉﻲﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪ‪ .‬ﺍﻟﻨﺎﺗﺞ ﺍﻟﻤﺤﻠﻲ ﺍﻹﺟﻤﺎﻟﻲ ﻭ ‪ = GTP‬ﻏﻮﺍﻧﻮﺯﻳﻦ ﺛﻨﺎﺉﻲ ﻭﺛﻼﺛﻲ ﺍﻟﻔﻮﺳﻔﺎﺕ ؛ ‪ = ADP‬ﺛﻨﺎﺉﻲ‬
‫ﻓﻮﺳﻔﺎﺕﺍﻷﺩﻳﻨﻮﺯﻳﻦ ؛ ﺹﺃﻧﺎ= ﻓﻮﺳﻔﺎﺕ ﻏﻴﺮ ﻋﻀﻮﻱ‪.‬‬
 ‫ﺃﺳﺉﻠﺔﺍﻟﺪﺭﺍﺳﺔ‬
‫ﺍﺧﺘﺮﺃﻓﻀﻞ ﺇﺟﺎﺑﺔ ﻭﺍﺣﺪﺓ‪.‬‬

‫‪ - 1.‬ﺗﺤﻮﻳﻞ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ ﺇﻟﻰ ﺃﻧﺰﻳﻢ ﺃﺳﻴﺘﻴﻞ ‪ A‬ﻭﺛﺎﻧﻲ ﺃﻛﺴﻴﺪ ﺍﻟﻜﺮﺑﻮﻥ‪:‬‬

‫‪.‬ﻳﻨﻄﻮﻱﻋﻠﻰ ﻣﺸﺎﺭﻛﺔ ﺣﻤﺾ ﻟﻴﺒﻮﻳﻚ ‪A.‬‬
‫ﻳﺘﻢﺗﻨﺸﻴﻂ ‪ .B‬ﻋﻨﺪﻣﺎ ﻳﺘﻢ ﻓﺴﻔﺮﺓ ﺑﻴﺮﻭﻓﺎﺕ ‪ decarboxylase‬ﻣﻦ ﻣﺮﻛﺐ ﻧﺎﺯﻋﺔ‬
‫ﻫﻴﺪﺭﻭﺟﻴﻦﺍﻟﺒﻴﺮﻭﻓﺎﺕ )‪ (PDHC‬ﺑﻮﺍﺳﻄﺔ ‪ PDH‬ﻛﻴﻨﺎﺯ ﻓﻲ ﻭﺟﻮﺩ ‪.ATP‬‬

‫ﺟﻴﻢﻗﺎﺑﻞ ﻟﻠﻌﻜﺲ‪.‬‬
‫‪.‬ﻳﺤﺪﺙﻓﻲ ﺍﻟﻌﺼﺎﺭﺓ ﺍﻟﺨﻠﻮﻳﺔ ‪D.‬‬
‫‪.‬ﻳﺘﻄﻠﺐﺃﻧﺰﻳﻢ ﺍﻟﺒﻴﻮﺗﻴﻦ ‪E.‬‬

‫ﺍﻹﺟﺎﺑﺔﺍﻟﺼﺤﻴﺤﺔ = ﺃ‪ .‬ﺣﻤﺾ ﻟﻴﺒﻮﻳﻚ ﻫﻮ ﻣﺘﻘﺒﻞ ﻭﺳﻴﻂ ﻟﻤﺠﻤﻮﻋﺔ ﺍﻷﺳﻴﺘﻴﻞ ﺍﻟﻤﺘﻜﻮﻧﺔ ﻓﻲ‬

‫ﺍﻟﺘﻔﺎﻋﻞ‪].‬ﻣﻼﺣﻈﺔ‪ :‬ﺣﻤﺾ ﻟﻴﺒﻮﻳﻚ ﺍﻟﻤﺮﺗﺒﻂ ﺑﺒﻘﺎﻳﺎ ﻟﻴﺴﻴﻦ ﻓﻲ ‪ E2‬ﻳﻌﻤﻞ ﻛـ "ﺫﺭﺍﻉ ﻣﺘﺄﺭﺟﺢ"‬
‫ﻳﺴﻤﺢﺑﺎﻟﺘﻔﺎﻋﻞ ﻣﻊ ‪ E1‬ﻭ ‪ [.E3‬ﻳﺤﻔﺰ ‪ PDHC‬ﺗﻔﺎﻋﻼً ﻻ ﺭﺟﻌﺔ ﻓﻴﻪ ﻳﺘﻢ ﺗﺜﺒﻴﺘﻪ ﻋﻨﺪﻣﺎ ﻳﺘﻢ ﻓﺴﻔﺮﺓ‬
‫ﻣﻜﻮﻥﺩﻳﻜﺎﺭﺑﻮﻛﺴﻴﻼﺯ )‪ .(E1‬ﻳﻘﻊ ‪ PDHC‬ﻓﻲ ﻣﺼﻔﻮﻓﺔ ﺍﻟﻤﻴﺘﻮﻛﻮﻧﺪﺭﻳﺎ‪ .‬ﻳﺘﻢ ﺍﺳﺘﺨﺪﺍﻡ ﺍﻟﺒﻴﻮﺗﻴﻦ‬
‫ﺑﻮﺍﺳﻄﺔﺍﻟﻜﺮﺑﻮﻛﺴﻴﻼﺯ ‪ ،‬ﻭﻟﻴﺲ ﻧﺰﻉ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ‪.‬‬

‫‪ .2.‬ﺃﻱ ﻣﻦ ﺍﻟﺤﺎﻻﺕ ﺍﻟﺘﺎﻟﻴﺔ ﺗﻘﻠﻞ ﻣﻦ ﺃﻛﺴﺪﺓ ﺃﻧﺰﻳﻢ ﺍﻷﺳﻴﺘﻴﻞ ﺃ ﺑﺪﻭﺭﺓ ﺣﺎﻣﺾ ﺍﻟﺴﺘﺮﻳﻚ؟‬

‫ﺗﻮﺍﻓﺮﺍﻟﻜﺎﻟﺴﻴﻮﻡ ﺑﻨﺴﺒﺔ ﻋﺎﻟﻴﺔ ‪A.‬‬

‫ﻧﺴﺒﺔﻋﺎﻟﻴﺔ ﻣﻦ ﺍﻷﺳﻴﺘﻴﻞ ‪CoA / CoA‬‬
‫ﺝ‪-‬ﻧﺴﺒﺔ ﻣﻨﺨﻔﻀﺔ ﻣﻦ ‪ATP / ADP‬‬

‫‪ NAD‬ﻣﻨﺨﻔﻀﺔ ‪ /+D. A‬ﻧﺴﺒﺔ ‪NADH‬‬

‫ﺍﻹﺟﺎﺑﺔﺍﻟﺼﺤﻴﺤﺔ = ‪+D. A low NAD‬ﻳﺘﺄﻛﺴﺪ ﻟﺘﻘﻠﻴﻞ ﺍﻟﻨﻴﻜﻮﺗﻴﻨﺎﻣﻴﺪ) ‪/ NADH‬‬

‫ﺛﻨﺎﺉﻲﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪ ﺍﻷﺩﻳﻨﻴﻦ( ﻳﺤﺪ ﻣﻦ ﻣﻌﺪﻻﺕ ‪ -+NAD‬ﻃﻠﺐ ﻧﺎﺯﻋﺎﺕ ﺍﻟﻬﻴﺪﺭﻭﺟﻴﻦ‪ .‬ﺇﻥ‬
‫ﺍﻟﺘﻮﺍﻓﺮﺍﻟﻌﺎﻟﻲ ﻟﻠﻜﺎﻟﺴﻴﻮﻡ ﻭﺍﻟﺮﻛﻴﺰﺓ )ﺃﻧﺰﻳﻢ ﺃﺳﻴﺘﻴﻞ ﺃ( ﻭﺍﻧﺨﻔﺎﺽ ﻧﺴﺒﺔ ‪) ATP / ADP‬ﺍﻷﺩﻳﻨﻮﺯﻳﻦ‬
‫ﺛﻼﺛﻲﺇﻟﻰ ﺛﻨﺎﺉﻲ ﺍﻟﻔﻮﺳﻔﺎﺕ( ﻳﺤﻔﺰ ﺍﻟﺪﻭﺭﺓ‪.‬‬

‫‪ .3.‬ﻣﺎ ﻳﻠﻲ ﻫﻮ ﻣﺠﻤﻮﻉ ﺛﻼﺙ ﺧﻄﻮﺍﺕ ﻓﻲ ﺩﻭﺭﺓ ﺣﺎﻣﺾ ﺍﻟﺴﺘﺮﻳﻚ‪ .‬ﺃ ‪ +‬ﺏ ‪+‬‬
‫ﻓﺎﺩ‪ +‬ﺡ‪+ NADH2O → C + FADH2‬‬
 ‫ﺍﺧﺘﺮﺍﻹﺟﺎﺑﺔ ﺫﺍﺕ ﺍﻟﺤﺮﻭﻑ ﺍﻟﺘﻲ ﺗﺘﻮﺍﻓﻖ ﻣﻊ ﺍﻷﺣﺮﻑ "ﺃ" ﻭ "ﺏ" ﻭ‬
‫"ﺝ" ﻓﻲ ﺍﻟﻤﻌﺎﺩﻟﺔ‪.‬‬

‫ﺍﻹﺟﺎﺑﺔﺍﻟﺼﺤﻴﺤﺔ = ‪+ NADH + FADH2+ FAD + H.+B. Succinate + NAD‬‬

‫‪.2O → oxaloacetate‬‬

‫‪ .4.‬ﺭﺟﻞ ﻳﺒﻠﻎ ﻣﻦ ﺍﻟﻌﻤﺮ ﺷﻬﺮ ﻭﺍﺣﺪ ﻳﻈﻬﺮ ﻋﻠﻴﻪ ﻣﺸﺎﻛﻞ ﻋﺼﺒﻴﺔ ﻭﺣﻤﺎﺽ ﻟﺒﻨﻲ‪ .‬ﺃﻇﻬﺮ ﻓﺤﺺ‬
‫ﺍﻹﻧﺰﻳﻢﻟﻨﺸﺎﻁ ﻣﺮﻛﺐ ﻧﺎﺯﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻦ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ )‪ (PDHC‬ﻋﻠﻰ ﻣﻘﺘﻄﻔﺎﺕ ﻣﻦ ﺍﻟﺨﻼﻳﺎ‬
‫ﺍﻟﻠﻴﻔﻴﺔﺍﻟﺠﻠﺪﻳﺔ ﺍﻟﻤﺰﺭﻭﻋﺔ ‪ ٪ 5‬ﻣﻦ ﺍﻟﻨﺸﺎﻁ ﺍﻟﻄﺒﻴﻌﻲ ﻣﻊ ﺗﺮﻛﻴﺰ ﻣﻨﺨﻔﺾ ﻣﻦ ﺑﻴﺮﻭﻓﻮﺳﻔﺎﺕ‬
‫ﺍﻟﺜﻴﺎﻣﻴﻦ)‪ (TPP‬ﻭﻟﻜﻦ ‪ ٪ 80‬ﻣﻦ ﺍﻟﻨﺸﺎﻁ ﺍﻟﻄﺒﻴﻌﻲ ﻋﻨﺪﻣﺎ ﺍﺣﺘﻮﻯ ﺍﻟﻔﺤﺺ ﻋﻠﻰ ﺗﺮﻛﻴﺰ‬
‫ﺃﻋﻠﻰﺑﺄﻟﻒ ﻣﺮﺓ ﻣﻦ ‪ . TPP‬ﺃﻱ ﻣﻦ ﺍﻟﻌﺒﺎﺭﺍﺕ ﺍﻟﺘﺎﻟﻴﺔ ﺍﻟﻤﺘﻌﻠﻘﺔ ﺑﻬﺬﺍ ﺍﻟﻤﺮﻳﺾ ﺻﺤﻴﺤﺔ؟‬

‫ﺝ‪:‬ﻣﻦ ﺍﻟﻤﺘﻮﻗﻊ ﺃﻥ ﻳﺆﺩﻱ ﺗﻨﺎﻭﻝ ﺍﻟﺜﻴﺎﻣﻴﻦ ﺇﻟﻰ ﺗﻘﻠﻴﻞ ﻣﺴﺘﻮﻯ ﺍﻟﻼﻛﺘﺎﺕ ﻓﻲ ﺍﻟﺪﻡ ﻭﺗﺤﺴﻴﻦ‬
‫ﺍﻷﻋﺮﺍﺽﺍﻟﺴﺮﻳﺮﻳﺔ ﻟﺪﻳﻪ‪.‬‬
‫ﺏ‪.‬ﻣﻦ ﺍﻟﻤﺘﻮﻗﻊ ﺃﻥ ﻳﻜﻮﻥ ﺍﺗﺒﺎﻉ ﻧﻈﺎﻡ ﻏﺬﺍﺉﻲ ﻋﺎﻟﻲ ﺍﻟﻜﺮﺑﻮﻫﻴﺪﺭﺍﺕ ﻣﻔﻴﺪﺍً ﻟﻬﺬﺍ ﺍﻟﻤﺮﻳﺾ‪.‬‬

‫ﺝ‪.‬ﻣﻦ ﺍﻟﻤﺘﻮﻗﻊ ﺯﻳﺎﺩﺓ ﺇﻧﺘﺎﺝ ﺍﻟﺴﻴﺘﺮﺍﺕ ﻣﻦ ﺍﻟﺘﺤﻠﻞ ﺍﻟﻬﻮﺍﺉﻲ‪.‬‬

‫ﻣﻦﺍﻟﻤﺘﻮﻗﻊ ﺃﻥ ﻳﻜﻮﻥ ‪ D. PDH‬ﻛﻴﻨﺎﺯ ‪ ،‬ﻭﻫﻮ ﺇﻧﺰﻳﻢ ﺗﻨﻈﻴﻤﻲ ﻟـ ‪ ، PDHC‬ﻧﺸﻄﺎً‪.‬‬

‫ﺍﻹﺟﺎﺑﺔﺍﻟﺼﺤﻴﺤﺔ = ﺃ‪ .‬ﻳﺒﺪﻭ ﺃﻥ ﺍﻟﻤﺮﻳﺾ ﻟﺪﻳﻪ ﺍﺳﺘﺠﺎﺑﺔ ﻟﻠﺜﻴﺎﻣﻴﻦ‬

‫ﻧﻘﺺ‪ .PDHC‬ﻳﻔﺸﻞ ﻣﻜﻮﻥ ﺑﻴﺮﻭﻓﺎﺕ ﺩﻳﻜﺎﺭﺑﻮﻛﺴﻴﻼﺯ )‪ (E1‬ﻓﻲ ‪ PDHC‬ﻓﻲ ﺭﺑﻂ‬
‫ﺑﻴﺮﻭﻓﻮﺳﻔﺎﺕﺍﻟﺜﻴﺎﻣﻴﻦ ﺑﺘﺮﻛﻴﺰ ﻣﻨﺨﻔﺾ ﻭﻟﻜﻨﻪ ﻳﻈﻬﺮ ﻧﺸﺎﻃﺎً ﻛﺒﻴﺮﺍً ﻋﻨﺪ ﺗﺮﻛﻴﺰ ﻋﺎﻝ ٍ‬
‫ﻣﻦﺍﻹﻧﺰﻳﻢ ﺍﻟﻤﺴﺎﻋﺪ‪ .‬ﻫﺬﻩ ﺍﻟﻄﻔﺮﺓ ﺍﻟﺘﻲ ﺗﺆﺛﺮ ﻋﻠﻰ ‪.K‬ﻡ)ﺛﺎﺑﺖ ﻣﻴﻜﺎﻳﻠﻴﺲ(‬

‫ﻣﻦﺇﻧﺰﻳﻢ ﺍﻹﻧﺰﻳﻢ ‪ ،‬ﻣﻮﺟﻮﺩ ﻓﻲ ﺑﻌﺾ ﺣﺎﻻﺕ ﻧﻘﺺ ‪ ، PDHC‬ﻭﻟﻜﻦ ﻟﻴﺲ ﻛﻠﻬﺎ‪ .‬ﻷﻥ‬
‫‪ PDHC‬ﺟﺰء ﻻ ﻳﺘﺠﺰﺃ ﻣﻦ‬
 ‫ﺍﺳﺘﻘﻼﺏﺍﻟﻜﺮﺑﻮﻫﻴﺪﺭﺍﺕ ‪ ،‬ﻣﻦ ﺍﻟﻤﺘﻮﻗﻊ ﺃﻥ ﻳﺆﺩﻱ ﺍﺗﺒﺎﻉ ﻧﻈﺎﻡ ﻏﺬﺍﺉﻲ ﻣﻨﺨﻔﺾ‬
‫ﺍﻟﻜﺮﺑﻮﻫﻴﺪﺭﺍﺕﺇﻟﻰ ﺗﺨﻔﻴﻒ ﺁﺛﺎﺭ ﻧﻘﺺ ﺍﻹﻧﺰﻳﻢ‪ .‬ﻳﻮﻟﺪ ﺗﺤﻠﻞ ﺍﻟﺴﻜﺮ ﺍﻟﻬﻮﺍﺉﻲ ﻣﺎﺩﺓ‬
‫ﺍﻟﺒﻴﺮﻭﻓﺎﺕ ‪،‬ﻭﻫﻲ ﺭﻛﻴﺰﺓ ‪ .PDHC‬ﺍﻧﺨﻔﺎﺽ ﻧﺸﺎﻁ ﺍﻟﻤﻌﻘﺪ ﻳﻘﻠﻞ ﻣﻦ ﺇﻧﺘﺎﺝ ﺃﻧﺰﻳﻢ‬
‫ﺍﻷﺳﻴﺘﻴﻞ‪ ، A‬ﻭﻫﻮ ﺭﻛﻴﺰﺓ ﻟﺘﺼﻨﻴﻊ ﺍﻟﺴﺘﺮﺍﺕ‪ .‬ﻧﻈﺮﺍً ﻷﻥ ‪ PDH kinase‬ﻳﺘﻢ ﺗﺜﺒﻴﻄﻪ‬
‫ﺑﻮﺍﺳﻄﺔﺍﻟﺒﻴﺮﻭﻓﺎﺕ ‪ ،‬ﻓﻬﻮ ﻏﻴﺮ ﻧﺸﻂ‪.‬‬

‫‪ .5.‬ﻣﺎ ﻫﻮ ﺍﻹﻧﺰﻳﻢ ﺍﻟﻤﺴﺎﻋﺪ ‪ -‬ﺍﻟﺮﻛﻴﺰﺓ ﺍﻟﺘﻲ ﺗﺴﺘﺨﺪﻣﻬﺎ ﻧﺎﺯﻋﺎﺕ ﺍﻟﻬﻴﺪﺭﻭﺟﻴﻦ ﻓﻲ ﻛﻞ ﻣﻦ ﺗﺤﻠﻞ ﺍﻟﺴﻜﺮ‬
‫ﻭﺩﻭﺭﺓﺣﻤﺾ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻠﻴﻚ؟‬
‫ﻧﻴﻜﻮﺗﻴﻨﺎﻣﻴﺪﺍﻷﺩﻳﻨﻴﻦ ﺛﻨﺎﺉﻲ ﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪ ﺍﻟﻤﺆﻛﺴﺪ )‪ (+NAD‬ﻳﺴﺘﺨﺪﻡ ﻣﻦ ﻗﺒﻞ‬
‫ﺇﻧﺰﻳﻢﻧﺎﺯﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻦ ﺍﻟﻐﻠﻴﺴﺮﺍﻟﺪﻳﻬﻴﺪ ‪-3‬ﻓﻮﺳﻔﺎﺕ ﻣﻦ ﺗﺤﻠﻞ ﺍﻟﺴﻜﺮ ﻭﺇﻳﺰﻭﺳﻴﺘﺮﺍﺕ‬
‫ﺩﻳﻬﻴﺪﺭﻭﺟﻴﻨﻴﺰ ‪،‬ﻭﻧﺰﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻨﻴﺰ ﺃﻟﻔﺎ ﻛﻴﺘﻮﺟﻠﻮﺗﺎﺭﺍﺕ ‪ ،‬ﻭﻧﺰﻋﺔ ﻫﻴﺪﺭﻭﺟﻴﻦ ﺍﻟﻤﺎﻻﺕ‬
‫ﻟﺪﻭﺭﺓﺣﻤﺾ ﺍﻟﻜﺮﺑﻮﻛﺴﻴﻞ‪] .‬ﻣﻼﺣﻈﺔ‪ :‬ﻳﺘﻄﻠﺐ ‪ E3‬ﻟﻤﺮﻛﺐ ﺍﻟﺒﻴﺮﻭﻓﺎﺕ ﺩﻳﻬﻴﺪﺭﻭﺟﻴﻨﻴﺰ‬
‫ﻓﻼﻓﻴﻦﺃﺩﻳﻨﻴﻦ ﻣﺆﻛﺴﺪ‬
‫ﺛﻨﺎﺉﻲﺍﻟﻨﻮﻛﻠﻴﻮﺗﻴﺪ )‪ (FAD‬ﻭ ‪[.+NAD‬‬