日期:
2020 年 — 2026 年
2020
2021
2022
2023
2024
2025
2026
影响因子:

Mechanism of action and impact of thiol homeostasis on efficacy of an enzyme replacement therapy for classical homocystinuria

硫醇稳态对经典同型半胱氨酸尿症酶替代疗法疗效的作用机制及影响

Philipp, Thilo Magnus; Bottiglieri, Teodoro; Clapper, Wilmelenne; Liu, Kai; Rodems, Steve; Szabo, Csaba; Majtan, Tomas

Catalytic specificity and crystal structure of cystathionine γ-lyase from Pseudomonas aeruginosa

铜绿假单胞菌胱硫醚γ-裂解酶的催化特异性和晶体结构

Pedretti, Marco; Fernández-Rodríguez, Carmen; Conter, Carolina; Oyenarte, Iker; Favretto, Filippo; di Matteo, Adele; Dominici, Paola; Petrosino, Maria; Martinez-Chantar, Maria Luz; Majtan, Tomas; Astegno, Alessandra; Martínez-Cruz, Luis Alfonso

Recent therapeutic approaches to cystathionine beta-synthase-deficient homocystinuria

胱硫醚β-合成酶缺乏型同型半胱氨酸尿症的最新治疗方法

Majtan, Tomas; Kožich, Viktor; Kruger, Warren D

H(2)S biogenesis by cystathionine beta-synthase: mechanism of inhibition by aminooxyacetic acid and unexpected role of serine.

胱硫醚β-合成酶的H(2)S生物合成:氨氧乙酸的抑制机制和丝氨酸的意外作用

Petrosino Maria, Zuhra Karim, Kopec Jola, Hutchin Andrew, Szabo Csaba, Majtan Tomas

Cystathionine-β-Synthase: Molecular Regulation and Pharmacological Inhibition

胱硫醚β-合成酶:分子调控和药理学抑制

Zuhra, Karim; Augsburger, Fiona; Majtan, Tomas; Szabo, Csaba

Hypermethioninemia Leads to Fatal Bleeding and Increased Mortality in a Transgenic I278T Mouse Model of Homocystinuria

高蛋氨酸血症导致转基因I278T小鼠同型半胱氨酸尿症模型发生致命性出血和死亡率增加

Park, Insun; Johnson, Linda K; Cox, Allaura; Branchford, Brian R; Di Paola, Jorge; Bublil, Erez M; Majtan, Tomas

Behavior, body composition, and vascular phenotype of homocystinuric mice on methionine-restricted diet or enzyme replacement therapy

蛋氨酸限制饮食或酶替代疗法对同型半胱氨酸尿症小鼠的行为、身体成分和血管表型的影响

Majtan, Tomas; Park, Insun; Cox, Allaura; Branchford, Brian R; di Paola, Jorge; Bublil, Erez M; Kraus, Jan P

Import of TAT-Conjugated Propionyl Coenzyme A Carboxylase Using Models of Propionic Acidemia.

利用丙酸血症模型研究TAT结合的丙酰辅酶A羧化酶的导入

Collard Renata, Majtan Tomas, Park Insun, Kraus Jan P

Enzyme replacement prevents neonatal death, liver damage, and osteoporosis in murine homocystinuria

酶替代疗法可预防小鼠同型半胱氨酸尿症的新生儿死亡、肝损伤和骨质疏松症。

Majtan, Tomas; Hůlková, Helena; Park, Insun; Krijt, Jakub; Kožich, Viktor; Bublil, Erez M; Kraus, Jan P

Domain organization, catalysis and regulation of eukaryotic cystathionine beta-synthases

真核生物胱硫醚β-合成酶的结构域组织、催化和调控

Majtan, Tomas; Pey, Angel L; Fernández, Roberto; Fernández, José A; Martínez-Cruz, Luis A; Kraus, Jan P