탈수소 효소 phosphatasePyruvate
Pyruvate dehydrogenase phosphatase| PDP1 | |||||||||||||||||||||||||||||||
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| 에일리어스 | PDP1, PDH, PDP, PDPC, PPM2C, PPM2A, 피루브산탈수소효소포스파타아제 촉매서브유닛1, 피루브산탈수소효소포스파타아제 촉매서브유닛1 | ||||||||||||||||||||||||||||||
| 외부 ID | OMIM: 605993 MGI: 2685870 HomoloGene: 31928 GeneCard: PDP1 | ||||||||||||||||||||||||||||||
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단백질 포스파타아제 2C로도 알려진 피루브산탈수소효소 촉매 서브유닛 1(PDPC 1)은 사람에게서 PDP1 [5][6]유전자에 의해 암호화되는 효소이다.PDPC 1은 피루브산탈수소효소에 대한 피루브산탈수소효소 인산화효소의 영향을 역전시켜 피루브산탈수소효소를 활성화시키는 효소이다.
기능.
피루브산탈수소효소(E1)는 대형 피루브산탈수소효소 복합체의 3가지 성분(E1, E2, E3) 중 하나이다.피루브산탈수소효소 키나아제는 E1의 세린 잔기의 인산화를 촉매하여 E1 성분을 비활성화시키고 복합체를 억제한다.피루브산탈수소효소 포스파타아제는 E1 성분의 탈인산화 및 활성화를 촉매하여 피루브산탈수소효소 키나아제 효과를 역전시킨다.
피루브산탈수소효소인산가수분해효소는 촉매 및 조절 서브유닛으로 구성된 헤테로다이머이다.두 개의 촉매 서브유닛이 보고되었다; 하나는 주로 골격근에서 발현되고 다른 하나는 간에서 훨씬 더 풍부하다.이 유전자에 의해 코드된 촉매 서브유닛은 전자이며 단백질 포스파타아제 2C(PP2C) 슈퍼패밀리에 속한다.피루브산탈수소효소복합체 및 피루브산탈수소효소 키나아제와 함께 이 효소는 미토콘드리아 [5]매트릭스에 위치한다.
규정
피루브산탈수소효소인산가수분해효소는 인슐린, PEP 및 AMP에 의해 자극되지만 ATP, NADH 및 아세틸-CoA에 의해 경쟁적으로 억제된다.
임상적 의의
이 유전자의 돌연변이는 피루브산탈수소효소 포스파타아제 [5][clarification needed]결핍증을 일으킨다.
레퍼런스
- ^ a b c GRCh38: 앙상블 릴리즈 89: ENSG00000164951 - 앙상블, 2017년 5월
- ^ a b c GRCm38: 앙상블 릴리즈 89: ENSMUSG000049225 - 앙상블, 2017년 5월
- ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
- ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
- ^ a b c "Entrez Gene: pyruvate dehydrogenase phosphatase catalytic subunit 1".
- ^ Lawson JE, Niu XD, Browning KS, Trong HL, Yan J, Reed LJ (Sep 1993). "Molecular cloning and expression of the catalytic subunit of bovine pyruvate dehydrogenase phosphatase and sequence similarity with protein phosphatase 2C". Biochemistry. 32 (35): 8987–93. doi:10.1021/bi00086a002. PMID 8396421.
추가 정보
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외부 링크
이 기사에는 미국 국립 의학 도서관(미국 국립 의학 도서관)의 공공 도메인 텍스트가 포함되어 있습니다.
