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Ubiquitin Ligases in Cholesterol Metabolism

Jiang W, Song BL

To maintain cholesterol homeostasis, the processes of cholesterol metabolism are regulated at multiple levels including transcription, translation, and enzymatic activity. Recently, the regulation of protein stability of some key players...
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Measurement of Malonate Concentration using Malonyl-CoA Synthetase and Its Relationship with Disease

Han SK, Kim JK, Park SR, Chang CS, Kim YS

  • KMID: 1661187
  • J Korean Pediatr Soc.
  • 1995 Sep;38(9):1167-1175.
No abstract available.
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The Ubiquitin-Proteasome System and F-box Proteins in Pathogenic Fungi

Liu TB, Xue C

  • KMID: 2312649
  • Mycobiology.
  • 2011 Dec;39(4):243-248.
The ubiquitin-proteasome system is one of the major protein turnover mechanisms that plays important roles in the regulation of a variety of cellular functions. It is composed of E1 (ubiquitin-activating...
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Ubiquitination of Aquaporin: in the kidney

Lee YJ, Kwon TH

Ubiquitination is known to be important for endocytosis and lysosomal degradation of aquaporin-2 (AQP2). Ubiquitin (Ub) is covalently attached to the lysine residue of the substrate proteins and activation and...
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Pja2 Inhibits Wnt/β-catenin Signaling by Reducing the Level of TCF/LEF1

Song Y, Lee S, Kim JR, Jho EH

Ubiquitination of proteins plays an essential role in various cellular processes, including protein degradation, DNA repair, and cell signaling pathways. Previous studies have shown that protein ubiquitination is implicated in...
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A Case of Citrullinemia Controlled by Diet and Arginine

Jeong EY, Jeon SS, Seo SS

  • KMID: 2099009
  • J Korean Soc Neonatol.
  • 1999 Nov;6(2):280-280.
Citrullinemia is an inborn error of urea cycle metabolism caused by deficiency of arginosuccinate synthetase. It is characterized by hyperammonemia and high citrulline level in serum, CSF and urine. The...
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A Case of Citrullinemia

Park DS, Kim DU, Moon SO, Lee IJ

  • KMID: 2142438
  • J Korean Pediatr Soc.
  • 1997 Apr;40(4):584-587.
Citrullinemia is one of the five urea cycle defects and is caused by argininosuccinic acid synthetase deficiency ; conversion of citrulline to argininosuccinic acid is blocked. Severe hyperammonemia typically develops...
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Tiul1 and TGIF are Involved in Downregulation of TGFbeta1-induced IgA Isotype Expression

Park KH, Nam EH, Seo GY, Seo SR, Kim PH

TGF-beta1 is well known to induce Ig germ-line alpha (GLalpha) transcription and subsequent IgA isotype class switching recombination (CSR). Homeodomain protein TG-interacting factor (TGIF) and E3-ubiquitin ligases TGIF interacting ubiquitin...
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Homozygous Exon 4 Deletion in Parkin Gene in a Korean Family with Autosomal Recessive Early Onset Parkinsonism

Kim JS, Lee KS, Kim YI, Lee KH, Kim HT

The gene responsible for autosomal recessive parkinsonism, parkin, has recently been identified on chromosome 6q. It has been shown to be mutated in Japanese and European families, most of whom...
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The Role of Tripartite Motif Family Proteins in TGF-β Signaling Pathway and Cancer

Lee HJ

TGF-β signaling plays a tumor suppressive role in normal and premalignant cells but promotes tumor progression during the late stages of tumor development. The TGF-β signaling pathway is tightly regulated...
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Two Cases of Antisynthetase Syndrome Combined with Mechanic's Hand

Kim CK, Yoo DH, Song YH, Jung JH, Noh SM, Oh YB, Jun JB, Jung SS, Lee IH, Bae SC, Kim SY

  • KMID: 2202527
  • J Korean Rheum Assoc.
  • 1999 Jun;6(2):178-184.
The antisynthetase syndrome is characterized by inflammatory myositis associated with intersititial lung disease (ILD), polyarthritis, mechanic's hand, and Raynaud's phenomenon, and usually with fever. The patients with these conditions have...
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The first successful live birth following preimplantation genetic diagnosis using PCR for type 1 citrullinemia

Cho JH, Kim CH, Lee KH, Jeon IK, Kim JM, Kang BM

Type 1 citrullinemia (CTLN1) is an autosomal recessive inherited metabolic disorder caused by anargininosuccinicnate synthetase deficiency. The patient was a 38-year-old Korean woman who is a carrier for CTLN1 and...
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Acute Respiratory Distress Syndrome as the Initial Clinical Manifestation of an Antisynthetase Syndrome

Kim SH, Park IN

Antisynthetase syndrome has been recognized as an important cause of autoimmune inflammatory myopathy in a subset of patients with polymyositis and dermatomyositis. It is associated with serum antibody to aminoacyl-transfer...
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The Effects of Glutathione on Cyclosporine Induced Cytotoxicity in Cultured Insulinoma Cells

Kim BC, Kim SH, Min YD, Chang JH

  • KMID: 2198971
  • J Korean Soc Transplant.
  • 2001 Dec;15(2):147-150.
PURPOSE: Cycloporine A (CsA) is a immunosuppressive agent most widely used in organ transplanted patients for preventing immunorejection, but it has some side effects, including hypertension, nephrotoxicity, hepatotoxicity and diabetes....
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The Change of Glutathione Content in the Placenta of Patients with Pregnancy-induced Hypertension

Han SJ, Kim WS

  • KMID: 2075307
  • Korean J Obstet Gynecol.
  • 1997 Sep;40(9):1871-1880.
Glutathione has a key role in several detoxification reactions and in the protection against injury caused by reactive oxygens. Pregnancy-induced hypertension(PIH) is associated with endothelial cell dysfunction. Such dysfunction could...
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A Case of Villous Adenoma with Depletion Syndrome

Choi NS, Kim HJ, Lee JH, Lee SH, Park CO, Lee WW, Dong SH, Kim BH, Chang YW, Lee JI, Chang R

  • KMID: 2115834
  • Korean J Gastrointest Endosc.
  • 1998 Feb;18(1):107-110.
Massive secretory diarrhea with pre-renal insufficiency, hyponatremia, hypokalemia and metabolic alkalosis or acidosis is associated with some large villous adenomas of the rectum and is called with depletion syndrome. This...
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Effects of Glutathione on Cisplatin-Induced Cytotoxicity In Human Cervical Cancer Cell Lines

Ahn TG, Han SJ

  • KMID: 1925511
  • Korean J Obstet Gynecol.
  • 2002 Aug;45(8):1289-1293.
OBJECTIVE: The purpose of this study is to determine the effects of glutathione on cisplatin-induced cytotoxicity of human cervical carcinoma cell lines (SiHa: squamous cell carcinoma cell, CaSki: epidermoid metastatic...
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A Case of Adult-type Citrullinemia with Hyperammonemia

Park HJ, Lim HJ, Jung IS, Kim YH, Kim IH, Chung IK, Kim HS, Park SH, Lee MH, Kim SJ, Lee DH

  • KMID: 2085022
  • Korean J Gastroenterol.
  • 2002 May;39(5):379-385.
Adult type citrullinemia is a urea cycle enzymopathy, which is characterized by hyperammonemia, high citrulline level in serum and decreased activity of argininosuccinic acid synthetase in liver biopsy. Clinical symptoms...
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Significance of Serum and Urinary Malonate Concentration in Patients with Traumatic Brain Injury

Kim DH, Ha YS, Park CO

  • KMID: 2018987
  • J Korean Neurosurg Soc.
  • 1997 Apr;26(4):471-475.
When the brain is damaged, lipid peroxidation and malonate concentration are increased. The malonate activates the NMDA receptor and induces neuronal toxicity. To investigate the importance of malonate concentration as...
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Pseudoxanthoma Elasticum Accompanied with Vitamin K-dependent Coagulation Factor Deficiency

Kim JY, Kim JS, Gye J, Namkoong S, Park BC, Kim M, Hong SP

  • KMID: 2088659
  • Korean J Dermatol.
  • 2013 Feb;51(2):131-134.
Pseudoxanthoma elasticum is a multiorgan disorder, characterized by ectopic mineralization of elastic fibers of skin, eyes, and cardiovascular system. The dermatologic manifestations include laxity of skin, as well as cutis...
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