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"glutathione synthetase deficiency"¿¡ ´ëÇÑ °Ë»ö °á°úÀÔ´Ï´Ù. °Ë»ö °á°ú º¸´Â µµÁß¿¡ Tab ۸¦ ´©¸£½Ã¸é °Ë»ö âÀÌ ¼±Åõ˴ϴÙ.
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¿µ¹® iron deficiency anemia ÇÑ±Û Ã¶°áÇ̺óÇ÷
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  ÀûÇ÷±¸ÀÇ ±â´ÉÀº »ê¼Ò¸¦ ¿î¹ÝÇϴµ¥ ÀÖ´Ù. ÀûÇ÷±¸ ¼Ó¿¡ »ê¼Ò¿Í °áÇÕÀ» ÇÏ¿© »ê¼Ò¸¦ ¿î¹ÝÇϴ Ç÷»ö¼Ò¶ó´Â ¹°ÁúÀÌ ÀÖ´Ù. Ã¶Àº ÀÌ Ç÷»ö¼ÒÀÇ Áß¿äÇÑ ºÎºÐÀ» ÀÌ·ç´Â °ÍÀ¸·Î Ã¶ÀÌ ¾øÀ¸¸é Ç÷»ö¼Ò°¡ ¸¸µé¾îÁú ¼ö°¡ ¾ø´Ù. Ç÷»ö¼Ò°¡ ¾øÀ¸¸é ¿ª½Ã ÀûÇ÷±¸µµ ¸¸µé¾îÁöÁö ¾ÊÀ¸¹Ç·Î Ã¼³»¿¡ Ã¶ÀÌ ºÎÁ·Çϸ頺óÇ÷ÀÌ »ý±ä´Ù. À̠ö°áÇ̼º ºóÇ÷Àº ºóÇ÷ÀÇ ¿øÀΠÁß¿¡¼­ °¡Àå ÈçÇÑ °ÍÀÌ´Ù(¾à 25%¸¦ Â÷ÁöÇÑ´Ù). Ã¶ÀúÀå·®ÀÇ ÀúÇÏ-°áÇÌ, Ç÷ûö³óµµÀÇ ÀúÇÏ, Æ®¶õ½ºÆä¸°·® »ó½Â, Æ®¶õ½ºÆä¸°Æ÷È­µµÀÇ ÀúÇÏ, Ç÷»ö¼Ò³óµµ ¶Ç´Â Ç츶ÅäÅ©¸®Æ®ÀÇ ÀúÇÏ, Àú»ö¼Ò¼º´ëÀûÇ÷±¸¸¦ Æ¯Â¡À¸·Î Çϴ ºóÇ÷·Î¼­, »ýü ³»¿¡¼­ Ã¶ÀÌ Àå±â¿¡ °ÉÃÄ °áÇ̵Ǹ砱נ¶§¹®¿¡ Ç÷»ö¼Ò »ý»ê °¨¼Ò¿¡ ÀÇÇØ ÀϾ´Ù. Ã¢ÀÚ¿¡¼­ÀǠöÈí¼ö·® ºÎÁ·, Ã¶ÀÇ ¼ö¿ä Áõ´ë(À¯¾Æ±â, »çÃá±â, ÀÓ½Å), Ã¶¼Ò½Ç°úÀ×(ÃâÇ÷)¿¡ ÀÇÇØ ÀϾ¸ç, Æ¯È÷ »çÃá±â¿¡¼­ Æó°æ±â±îÁöÀÇ ¿©¼º¿¡°Ô ¸¹´Ù. Áõ»óÀ¸·Î¼­´Â ¾ó±¼Ã¢¹é, ÇǷΰ¨, ÇǺÎâ¹é, ¼ÕÅé º¯È­(½ºÇ¬ ¸ð¾ç) µîÀ» ³ªÅ¸³½´Ù. ±¸°­ ¿µ¿ª¿¡¼­´Â ÇôÀÇ Á¢ÃËÅë, ¹ßÀû, °ÇÁ¶°¨, »ïÅ´°ï¶õÀ» ¼ö¹ÝÇϸé Ç÷¯¸Ó-ºó½¼(Plummer-Vinson)ÁõÈıºÀ̶ó°í ÇÑ´Ù. Ç÷¾× ¼Ò°ßÀº Ç÷ûöÀº ÀúÇÏÇϸç, Ã¶°áÇÕ´É·ÂÀÇ »ó½Â, Àú»ö¼Ò¼º ÀÛÀºÀûÇ÷±¸¼ºÀ» ³ªÅ¸³½´Ù.
´ëÇÑÀÇÇù ÀÇÇпë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • glutathione synthetase
    ±Û·çŸƼ¿ÂÇÕ¼ºÈ¿¼Ò
  • synthetase
    ÇÕ¼ºÈ¿¼Ò
  • glutathione
    ±Û·çŸƼ¿Â
  • glutathione peroxidase
    ±Û·çŸƼ¿Â°ú»êÈ­È¿¼Ò
  • acquired immune deficiency syndrome
    ÈÄõ¸é¿ª°áÇÌÁõÈıº, ¿¡ÀÌÁî
  • biotin deficiency syndrome
    ºñ¿Àƾ°áÇÌÁõÈıº
  • deficiency
    1. °áÇÌ 2. °áÇÌÁõ
  • deficiency disease
    °áÇ̺´
  • erythropoietin deficiency anemia
    ¿¡¸®Æ®·ÎÆ÷ÀÌ¿¡Æ¾°áÇ̺óÇ÷
  • fat deficiency disease
    Áö¹æ°áÇ̺´
  • folate deficiency anemia
    ¿±»ê°áÇ̺óÇ÷
  • iron deficiency anemia
    ö°áÇ̺óÇ÷
  • immune deficiency
    ¸é¿ª°áÇÌ
  • leukocyte adhesion deficiency
    ¹éÇ÷±¸ºÎÂø°áÇÌ
  • nutritional deficiency disease
    ¿µ¾ç°áÇ̺´
´ëÇÑÀÇÇù Çʼö ÀÇÇпë¾îÁý »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 4 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • glutathione
    ±Û·çŸƼ¿Â
  • synthetase
    ÇÕ¼ºÈ¿¼Ò
  • iron deficiency anemia
    ö°áÇ̺óÇ÷
  • deficiency
    °áÇÌ(Áõ)
¿¾ ´ëÇÑÀÇÇù ÀÇÇпë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • synthetase
    ÇÕ¼ºÈ¿¼Ò
  • glutathione
    ±Û·çŸƼ¿Â
  • glutathione peroxidase
    ±Û·çŸƼ¿Â°ú»êÈ­È¿¼Ò
  • acid lipase deficiency
    »ê¼ºÁöÁúºÐÇØÈ¿¼Ò°áÇÌ
  • acquired immune deficiency
    ÈÄõ¸é¿ª°áÇÌ
  • acquired immune deficiency syndrome
    ÈÄõ¸é¿ª°áÇÌÁõÈıº, ¿¡ÀÌÁî
  • adhesion deficiency disorder
    À¯Âø°áÇÌÀå¾Ö
  • antibody deficiency syndrome
    Ç×ü°áÇÌÁõÈıº
  • erythropoietin deficiency anemia
    ¿¡¸®Æ®·ÎÆ÷¿¡Æ¾°áÇ̺óÇ÷
  • folate deficiency anemia
    ¿±»ê°áÇÌ
  • iron deficiency anemia
    ö°áÇ̺óÇ÷
  • ceruloplasmin deficiency
    ¼¼·ê·ÎÇö󽺹ΰáÇÌ
  • complement deficiency
    µµ¿òü°áÇÌ
  • deficiency
    °áÇÌ(Áõ)
  • deficiency disease
    ¿µ¾ç°áÇ̺´
¿¾ ´ëÇÑÀÇÇù 2 ÀÇÇпë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • glutathione reductase deficiency
    ±Û·çŸƼ¿Â ȯ¿øÈ¿¼Ò °áÇÌÁõ.
  • antibody to histidyl tRNa synthetase
    È÷½ºÆ¼µô tRNa ÇÕ¼ºÈ¿¼Ò¿¡ ´ëÇÑ Ç×ü
  • gamma-glutamyl cysteine synthetase
    °¨¸¶-±Û·çŸ¹Ð½Ã½ºÅ×ÀÎÇÕ¼ºÈ¿¼Ò
  • heme synthetase
    ÈûÇÕ¼ºÈ¿¼Ò.
  • heme synthetase
    ÇðÇÕ¼ºÈ¿¼Ò.
  • phosphoribosyl pyrophosphate synthetase
    Æ÷½ºÆ÷¸®º¸½ÇÇÇ·ÎÆ÷½ºÆäÀÌÆ®½ÅÅןÁ¦<ÇÕ¼ºÈ¿¼Ò>
  • glutathione
    ±Û·çŸƼ¿Â.
  • glutathione
    ±Û·çŸƼ¿Â
  • glutathione peroxidase
    ±Û·çŸƼ¿Â Æä¸£¿Á½Ã´ÙÁ¦<°ú»êÈ­È¿¼Ò>.
  • glutathione peroxidase
    ±Û·çŸƼ¿ÂÆä¸£¿Á½Ã´ÙÁ¦(°ú»êÈ­ È¿¼Ò)
  • glutathione peroxidase
    ±Û·çŸƼ¿ÂÆä¸£¿Á½Ã´ÙÁ¦<--°ú»êÈ­ È¿¼Ò>
  • glutathione reductase
    ±Û·çŸƼ¿Â ¸®´ÚŸÁ¦<ȯ¿øÈ¿¼Ò>.
  • glutathione reductase
    ±Û·çŸƼ¿Â¸®´ÚŸÁ¦<--ȯ¿øÈ¿¼Ò>
  • Acquiered immune deficiency syndrome
    ÈÄõ¼º ¸é¿ª °áÇÌÁõÈıº
  • Fluorine deficiency
    ºÒ¼Ò°áÇÌÁõ(Ý×áÈÌÀù¹ñø)
¿¾ ´ëÇÑÀÇÇù 3 ÀÇÇпë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • glutathione synthetase
    ±Û·çŸƼ¿ÂÇÕ¼ºÈ¿¼Ò
  • glutathione reductase deficiency
    ±Û·çŸƼ¿Â ȯ¿øÈ¿¼Ò °áÇÌÁõ.
  • glutathione
    ±Û·çŸƼ¿Â
  • glutathione
    ±Û·çŸƼ¿Â.
  • glutathione peroxidase
    ±Û·çŸƼ¿ÂÆä¸£¿Á½Ã´ÙÁ¦<--°ú»êÈ­ È¿¼Ò>
  • glutathione peroxidase
    ±Û·çŸƼ¿Â Æä¸£¿Á½Ã´ÙÁ¦<°ú»êÈ­È¿¼Ò>.
  • glutathione peroxidase
    ±Û·çŸƼ¿ÂÆä¸£¿Á½Ã´ÙÁ¦(°ú»êÈ­ È¿¼Ò)
  • glutathione reductase
    ±Û·çŸƼ¿Â¸®´ÚŸÁ¦<--ȯ¿øÈ¿¼Ò>
  • glutathione reductase
    ±Û·çŸƼ¿Â ¸®´ÚŸÁ¦<ȯ¿øÈ¿¼Ò>.
  • antibody to histidyl tRNa synthetase
    È÷½ºÆ¼µô tRNa ÇÕ¼ºÈ¿¼Ò¿¡ ´ëÇÑ Ç×ü
  • cystathionine synthetase
    ½Ã½ºÅ¸Æ¼¿À´ÑÇÕ¼ºÈ¿¼Ò(¡­ùêà÷ý£áÈ).
  • delta-aminolevulinic acid synthetase
    µ¨Å¸-¾Æ¹Ì³ë·¹ºÒ¸°»êÇÕ¼ºÈ¿¼Ò
  • gamma-glutamyl cysteine synthetase
    °¨¸¶-±Û·çŸ¹Ð½Ã½ºÅ×ÀÎÇÕ¼ºÈ¿¼Ò
  • heme synthetase
    ÇðÇÕ¼ºÈ¿¼Ò.
  • heme synthetase
    ÈûÇÕ¼ºÈ¿¼Ò.
´ëÇÑÇØºÎÇÐȸ ÀÇÇпë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • Deficiency (Nanismus)
    °áÇÌ(³­ÀåÀÌÁõ)
    [¿¾ ¿ë¾î] °áÇÌ
  • Organogenetic deficiency
    ±â°ü¹ß»ý°áÇÌ
    [¿¾ ¿ë¾î] ±â°ü¹ß»ý°áÇÌ
  • Functional deficiency
    ±â´É°áÇÌ
    [¿¾ ¿ë¾î] ±â´ÉÀû°áÇÌ
  • Pituitary hormone deficiency (Pituitary dwarfism)
    ³úÇϼöüȣ¸£¸ó°áÇÌ (³úÇϼöü³­ÀåÀÌ)
    [¿¾ ¿ë¾î] ³úÇϼöüȣ¸£¸ó°áÇÌ (³úÇϼöü³­ÀåÀÌ)
  • Reaction deficiency
    ¹ÝÀÀ°áÇÌ
    [¿¾ ¿ë¾î] ¹ÝÀÀ°áÇÌ
  • Vitamin deficiency (Fetal osteodystrophy)
    ºñŸ¹Î°áÇÌ(žƻÀ¿µ¾çÀå¾Ö)
    [¿¾ ¿ë¾î] ºñŸ¹Î°áÇÌ(žƻÀ¿µ¾çÀå¾Ö)
  • Cellular deficiency (Acallosal cerebrum)
    ¼¼Æ÷°áÇÌ (³úµéº¸°á¿©³ú)
    [¿¾ ¿ë¾î] ¼¼Æ÷°áÇÌ
  • Intracellular deficiency (Albinism)
    ¼¼Æ÷¼Ó°áÇÌ (¹é»öÁõ)
    [¿¾ ¿ë¾î] ¼¼Æ÷³»°áÇÌ
  • Chromosomal deficiency
    ¿°»öü°áÇÌ
    [¿¾ ¿ë¾î] ¿°»öü°á½Ç
  • Stimulation deficiency
    ÀڱذáÇÌ
    [¿¾ ¿ë¾î] ÀڱذáÇÌ
  • Sensory deficiency
    °¨°¢°áÇÌ
    [¿¾ ¿ë¾î] °¨°¢°áÇÌ
  • Thyroid hormone deficiency (Cretinism)
    °©»ó»ùÈ£¸£¸ó°áÇÌ (°©»ó»ù³­ÀåÀÌ)
    [¿¾ ¿ë¾î] °©»ó¼±È£¸£¸ó°áÇÌ (°©»ó»ù³­ÀåÀÌ)
  • Deficiency
    °áÇÌ
    [¿¾ ¿ë¾î] °áÇÌ
  • Deficiency (Monstrous tumor)
    °áÇÌ (±«¹°Á¾)
    [¿¾ ¿ë¾î] °áÇÌ
  • Secretion deficiency
    ºÐºñ°áÇÌ
    [¿¾ ¿ë¾î] ºÐºñ°áÇÌ
´ëÇÑ»ýÈ­ÇкÐÀÚ»ý¹°ÇÐȸ ¿ë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • glutathione
    ±Û·çŸƼ¿Â
  • glutathione-S-transferase
    ±Û·çŸƼ¿Â-S-Æ®·£½ºÆÛ·¹À̽º
  • aminoacyl-tRNA synthetase
    ¾Æ¹Ì³ë¾Æ½ÇtRNA ½ÅÅ×Å×À̽º
  • amylose synthetase(synthase)
    "¾Æ¹Ð·Î½º½ÅÅ×Å×À̽º, ¾Æ¹Ð·Î½º½ÅÅ×À̽º"
  • conditioned vitamin deficiency
    Á¶°ÇºÎ(ðÉËìݾ) ºñŸ¹Î°áÇË(ÌÀù¹)
  • deficiency
    °áÇÌ(ÌÀù¹)
  • deficiency disease
    °áÇÌ Áúȯ(ÌÀù¹òðü´)
  • deficiency mutant
    "°áÇÌ º¯ÀÌü(ÌÀù¹Ü¨ì¶ô÷), (ÔÒ) auxotroph"
  • dietary deficiency
    ½ÄÀ̰áÇÌ(ç½å×ÌÀù¹)
  • familial high-density lipoprotein deficiency °¡Á·¼º °í¹Ðµµ ÁöÁú´Ü¹éÁú °áÇÌ (Ê«ðéàõÍÔÚËÓøò·òõ
    Ó±ÛÜòõÌÀù¹)
  • familial lysosomal lipase deficiency
    °¡Á·¼º(Ê«ðéàõ) ¶óÀÌ¼Ò¼Ø ¶óÀÌÆäÀ̽º °áÇÌ(ÌÀù¹)
  • fatty acid synthetase system
    Áö¹æ»ê ÇÕ¼º È¿¼Ò(ò·Û¸ß«ùêà÷ý£áÈ)½Ã½ºÅÛ
  • glucose-6-phosphate dehydrogenase deficiency
    ±Û·çÄÚ½º-6-Àλê(×òß«) µðÇÏÀ̵å·ÎÀú³×À̽º °áÇÌ(ÌÀ
  • heme synthetase
    Èû½ÅÅ×Å×À̽º
  • lactose synthetase
    ¶ôÅ佺 ½ÅÅ×Å×À̽º
KI ÀÇÇпë¾î »çÀü °Ë»ö À¯»ç °Ë»ö °á°ú : 6 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
  • deficiency
    °áÇÌÁõ
  • deficiency anemia
    °áÇ̼ººóÇ÷
  • iron deficiency anemia
    ö°áÇ̼ººóÇ÷
  • mineral deficiency
    ¹«±âÁú°áÇÌ(Áõ)
  • nutritional deficiency disease
    ¿µ¾ç°áÇÌÁõ
  • vitamin deficiency
    ºñŸ¹Î°áÇÌ(Áõ)
KMLE ÀÇÇоà¾î »çÀü À¯»ç °Ë»ö °á°ú : 5 ÆäÀÌÁö: 1
GSD genetically significant dose; Gerstmann-Straussler disease; glutathione synthetase deficiency; glyco...
MD Doctor of Medicine [Lat. Medicinae Doctor]; magnesium deficiency; main duct; maintenance dose; major...
IGD idiopathic growth hormone deficiency; interglobal distance; isolated gonadotropin deficiency
MCD magnetic circular dichroism; mast-cell degranulation; mean cell diameter; mean of consecutive differ...
E-GR erythrocyte glutathione reductase
KMLE ÀÚµ¿ÃßÃâ ÀÇÇоà¾î »çÀü À¯»ç °Ë»ö °á°ú : 5 ÆäÀÌÁö: 1
2-5A synthetase 2', 5'-oligoadenylate synthetase
DCVG 1,2-dichlorovinyl glutathione
DNP-SG 2,4-dinitrophenyl S-glutathione
alpha-GST Alpha glutathione S-transferase
GST EA)-glutathione-S:- transferase
°æºÏ´ë Ä¡°ú´ëÇÐ ±¸°­³»°ú ±³½Ç »çÀü À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
  • ¿µ¹®
    ÇѱÛ
    ¼³¸í
  • synthetase
    ½ÅÅ×Å×À̽º, ½ÅÅןÁ¦, ÇÕ¼º È¿¼Ò
    ATP ȤÀº ±×¿Í À¯»çÇÑ »ïÀλ꿰¿¡¼­
  • 17-hydroxylase deficiency
    17-hydroxylase °áÇÌ
  • acquired immune deficiency syndrome
    ÈÄõ¼º ¸é¿ª °áÇÌ ÁõÈıº
    1. ÇöÀúÇÑ ¸é¿ª °áÇ̰ú ÇÔ²² ±âȸ°¨¿°, ¼Ó¹ß¼º ¾Ï ¹× ½Å°æ°è Áõ¼¼°¡ µ¿¹Ý. ¹ÙÀÌ·¯½º ÀÚü¿¡ ÀÇÇÑ º´º¯°ú ¸é¿ª´É·Â ÀúÇÏ¿¡ µû¸¥ ±âȸ °¨¿° µîÀÇ ÀÌÂ÷Àû º´º¯ÀÇ µÎ °¡Áö·Î ´ëº°. HIV¿¡ ÀÇÇØ ¹ß»ýµÇ´Â ÁúȯÀ¸·Î ½Å°æ°è°¡ Áß¿ä Ç¥ÀûÁß Çϳª. ¹ÙÀÌ·¯½º¿¡ °¨¿°µÈ »ç¶÷ÀÇ 40% Á¤µµ°¡ Áúº´ÀÌ ¹ß»ý. ¹ÙÀÌ·¯½ºÀÇ Á÷Á¢ÀûÀÎ ¿µÇâ¿¡ ÀÌÇÑ º´º¯À¸·Î´Â ¸²ÇÁ±¸¼º ¼ö¸·¿°°ú HIV ³ú¿° µîÀÌ ÀÖÀ½. 2. ÈÄõ¼º ¸é¿ª°áÇÌÁõ. Àΰ£ ¸é¿ª°áÇÌ ¹ÙÀÌ·¯½º
  • acquired immune deficiency syndrome
    ÈÄõ¼º ¸é¿ª°áÇÌ ÁõÈıº
  • adenosine deficiency
    ¾Æµ¥³ë½Å °áÇÌÁõ
  • ascorbic acid deficiency
    ¾Æ½ºÄÚ¸£ºó»ê °áÇÌÁõ
    Ư¡ÀûÀÎ ±«Ç÷º´ÀÌ ³ªÅ¸³ª¸ç ÀÌÀÇ Áõ»óÀ¸·Î´Â ÀÕ¸öÀÌ º×°í ½±°Ô ÃâÇ÷ÀÌ µÇ°í, Ä¡¾Æ Çü¼º Àå¾Ö Ä¡Á¶°ñ Èí¼ö ÇÇÇÏ ÃâÇ÷ µîÀÌ ÀÖÀ¸¸ç â»ó Ä¡À¯°¡ ´Ê¾îÁø´Ù.
  • cell adhesion molecular deficiency
    ¼¼Æ÷ À¯Âø ºÐÀÚ °áÇÌ
  • cellular deficiency
    ¼¼Æ÷ °áÇÌ
    ¼¼Æ÷°¡ À¯ÀüÀû ȤÀº ÀÚ°¡¸é¿ªÀû ¿äÀÎÀ¸·Î ÀÎÇØ¼­ °áÇÌµÈ °Í.
  • chromosomal deficiency
    ¿°»öü °áÇÌ
  • color deficiency
    »ö °áÇÌ
  • deficiency anemia
    °áÇ̼º ºóÇ÷
    Ç÷»ö¼Ò °áÇÌÀ¸·Î ÀÎÇÑ ºóÇ÷.
  • deficiency symptom
    °áÇÌ Áõ»ó
    ³»ºÐºñ¼±ÀÇ ºÐºñ Àå¾Ö¿¡ ÀÇÇÑ Áõ»ó.
  • diphosphatase deficiency
    µðÆ÷½ºÆÄŸÁ¦ °áÇÌ
  • electrolyte deficiency syndrome
    ÀüÇØÁú °áÇÌ ÁõÈıº
  • erythropoietin deficiency anemia
    ¿¡¸®Æ®·ÎÆ÷¿¡Æ¾ °áÇ̼º ºóÇ÷, ÀûÇ÷±¸ Á¶Ç÷ ÀÎÀÚ °áÇ̼º ºóÇ÷
CancerWEB ¿µ¿µ ÀÇÇлçÀü ¸ÂÃã °Ë»ö °á°ú : 1 ÆäÀÌÁö: 1
glutathione synthetase deficiency An inborn error of metabolism associated with massive urinary excretion of 5-oxyproline, elevated levels of 5-oxyproline in the blood and cerebrospinal fluid, severe metabolic acidosis, tendency toward haemolysis, and defective central nervous systems function. Glutathione synthetase deficiency has been reported as a generalised condition or with a deficiency restricted to erythrocytes.
(05 Mar 2000)
CancerWEB ¿µ¿µ ÀÇÇлçÀü À¯»ç °Ë»ö °á°ú : 15 ÆäÀÌÁö: 1
glutathione synthetase <enzyme> An enzyme that catalyses the formation of glutathione, ADP, and orthophosphate from gamma-glutamylcysteine, ATP, and glycine; a deficiency will lead to metabolic acidosis and progressive brain dysfunction.
(05 Mar 2000)
carbamoylphosphate synthetase deficiency <biochemistry> Carbamoylphosphate synthetase is the initial enzyme of the urea cycle, catalysing the synthesis of carbamoylphosphate from ammonia, bicarbonate and ATP as the first step of ammonia detoxification.
The enzyme is an intramitochondrial form called CPS I. A different isozyme found in the cytoplasm, called CPS II, is much less active and apparently not involved in the urea cycle. The deficiency state is autosomal recessive and presents in infancy with massive hyperammonaemia and neurologic deficits in survivors.
Diagnosis is suggested by the blood biochemistry and confirmed by specific enzyme assay on liver or rectal biopsy. Prenatal diagnosis by molecular methods has been used successfully in informative families.
Inheritance: autosomal recessive.
(07 Apr 1998)
reduced glutathione Glutathione acting as a hydrogen donor.
(05 Mar 2000)
glutathione <biochemistry> The tripeptide _ glutamylcysteinylglycine. It contains an unusual peptide linkage between the _ carboxyl group of the glutamate side chain and the amine group of cysteine.
The concentration of glutathione in animal cells is _5mM and its sulphydryl group is kept largely in the reduced state. This allows it to act as a sulphydryl buffer, reducing any disulphide bonds formed within cytoplasmic proteins to cysteines. Hence, few, if any, cytoplasmic proteins contain disulphide bonds.
Glutathione is also important as a cofactor for the enzyme glutathione peroxidase, in the uptake of amino acids and participates in leucotriene synthesis.
(18 Nov 1997)
glutathione disulfide <chemical> A glutathione derivative that forms when the sulfhydryl side chains of the cysteine residues of two glutathione molecules form a disulfide bond during the course of being oxidised with various oxides and peroxides in cells. Glutathione reductase, with the coupled oxidation of NADPH, reduces gssg to two moles of glutathione.
Chemical name: Bis(gamma-Glutamyl-L-cysteinylglycine) Disulfide
(12 Dec 1998)
glutathione oxidase <enzyme> Oxygen-dependent conversion of glutathione to glutathione disulfide
Registry number: EC 1.8.4.-
Synonym: renal thiol oxidase, renal sulfhydryl oxidase, intestinal thiol oxidase, kidney thiol oxidase
(26 Jun 1999)
glutathione peroxidase <enzyme> A detoxifying enzyme that eliminates hydrogen peroxide and organic peroxides.
Glutathione is an essential cofactor for the enzyme and its reaction involves the oxidation of glutathione (GSH) to glutathione disulphide (GSSG). The GSSG is then reduced to GSH by glutathione reductase. Glutathione peroxidase, (GPX), has a selenocysteine residue in its active site. Three forms of the enzyme exist: cy toplasmic GPX, plasma GPX and phospholipid hydroperoxide GPX.
(18 Nov 1997)
glutathione reductase <enzyme> An FAD containing enzyme, a dimer of 50 kD subunits.
It catalyses the NADP dependent reduction of glutathione disulphide (GSSG) to glutathione (GSH). This is an essential reaction that maintains a GSH:GSSG ratio in the cytoplasm of _500:1.
(18 Nov 1997)
glutathione S-transferase A class of enzymes that catalyze the reaction of glutathione with an acceptor molecule (e.g., an arene oxide) to form an S-substituted glutathione; a key step in detoxification of many substances; start of the mercapturic acid pathway.
Synonym: ligandin.
(05 Mar 2000)
glutathione synthase <enzyme> One of the enzymes active in the gamma-glutamyl cycle. It catalyses the synthesis of glutathione from gamma-glutamylcysteine and glycine in the presence of ATP with the formation of ADP and orthophosphate.
Chemical name: gamma-L-Glutamyl-L-cysteine:glycine ligase (ADP-forming)
Registry number: EC 6.3.2.3
(12 Dec 1998)
glutathione transferase <enzyme> A transferase that catalyses the addition of aliphatic, aromatic, or heterocyclic radicals as well as epoxides and arene oxides to glutathione. Addition takes place at the sulfur atom. It also catalyses the reduction of polyol nitrate by glutathione to polyol and nitrite.
Chemical name: RX:glutathione R-transferase
Registry number: EC 2.5.1.18
(12 Dec 1998)
phospholipid-hydroperoxide glutathione peroxidase <enzyme> Selenoenzyme found in biological materials; different from glutathione peroxidase EC 1.11.1.9
Registry number: EC 1.11.1.-
Synonym: pH-gperoxidase
(26 Jun 1999)
protein disulfide reductase (glutathione) <enzyme> An enzyme that catalyses the reduction of a protein-disulfide in the presence of glutathione, forming a protein-dithiol. Insulin is one of its substrates.
Chemical name: Glutathione:protein-disulfide oxidoreductase
Registry number: EC 1.8.4.2
(12 Dec 1998)
S-(dinitrophenyl)glutathione ATPase <enzyme> Anionic conjugates of bilirubin and bile acids stimulate the hydrolysis of the above enzyme of human erythrocyte; also found in other tissue
Registry number: EC 3.6.1.-
Synonym: dnp-sg-atpase
(26 Jun 1999)
sulfobromophthalein-glutathione conjugase <enzyme> Similar to EC 2.5.1.18; non-microsomal enzyme involving transfer of the dye from plasma to hepatic parenchymal cells, cellular storage, enzymatic intracellular conjugation with reduced glutathione and rate limited excretion into the bile; activity of this enzyme is used to determine conjugating ability of the liver
Registry number: EC 2.5.1.-
Synonym: bromsulphthalein-glutathione conjugating enzyme
(26 Jun 1999)
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