scholarly journals Liver neoplasms in methylmalonic aciduria: An emerging complication

2019 ◽  
Vol 42 (5) ◽  
pp. 793-802 ◽  
Author(s):  
Patrick Forny ◽  
Michel Hochuli ◽  
Yusof Rahman ◽  
Maesha Deheragoda ◽  
Achim Weber ◽  
...  
Keyword(s):  
Liver Neoplasms ◽  
Methylmalonic Aciduria

scholarly journals The Value of 1H-MRS and MRI in Combined Methylmalonic Aciduria and Homocystinuria

2019 ◽  
Vol 43 (4) ◽  
pp. 559-562
Author(s):  
Ailan Cheng ◽  
Rong Yao ◽  
Wenjun Cao ◽  
Hong Yu
Keyword(s):  
Methylmalonic Aciduria ◽  
1H Mrs

In vitro “responsive” methylmalonic aciduria: A new variant

1974 ◽  
Vol 84 (6) ◽  
pp. 911-912
Author(s):  
C. Kaye ◽  
G. Morrow ◽  
H. Nadler
Keyword(s):  
Methylmalonic Aciduria ◽  
New Variant

Hepatoblastoma in a patient with methylmalonic aciduria

2015 ◽  
Vol 167 (3) ◽  
pp. 635-638 ◽  
Author(s):  
Randall Chan ◽  
Leo Mascarenhas ◽  
Richard G Boles ◽  
Nanda Kerkar ◽  
Yuri Genyk ◽  
...  
Keyword(s):  
Methylmalonic Aciduria

New SUCLG1 patients expanding the phenotypic spectrum of this rare cause of mild methylmalonic aciduria

Mitochondrion ◽  
2010 ◽  
Vol 10 (4) ◽  
pp. 335-341 ◽  
Author(s):  
Vassili Valayannopoulos ◽  
Coralie Haudry ◽  
Valérie Serre ◽  
Magalie Barth ◽  
Nathalie Boddaert ◽  
...  
Keyword(s):  
Methylmalonic Aciduria ◽  
Phenotypic Spectrum

Liver neoplasms and the oral contraceptives

1977 ◽  
Vol 128 (4) ◽  
pp. 448-454 ◽  
Author(s):  
Walter S. Keifer ◽  
John C. Scott
Keyword(s):  
Oral Contraceptives ◽  
Liver Neoplasms

scholarly journals The human B12 trafficking protein CblC processes nitrocobalamin

2020 ◽  
Vol 295 (28) ◽  
pp. 9630-9640 ◽  
Author(s):  
Romila Mascarenhas ◽  
Zhu Li ◽  
Carmen Gherasim ◽  
Markus Ruetz ◽  
Ruma Banerjee
Keyword(s):  
Nitrite Reductase ◽  
Reductase Activity ◽  
Methylmalonic Aciduria ◽  
Axial Ligand ◽  
Vitamin B ◽  
Early Step ◽  
Trafficking Pathway ◽  
Nitrite Reductase Activity ◽  
Futile Cycling ◽  
Thiol Oxidase

In humans, cobalamin or vitamin B12 is delivered to two target enzymes via a complex intracellular trafficking pathway comprising transporters and chaperones. CblC (or MMACHC) is a processing chaperone that catalyzes an early step in this trafficking pathway. CblC removes the upper axial ligand of cobalamin derivatives, forming an intermediate in the pathway that is subsequently converted to the active cofactor derivatives. Mutations in the cblC gene lead to methylmalonic aciduria and homocystinuria. Here, we report that nitrosylcobalamin (NOCbl), which was developed as an antiproliferative reagent, and is purported to cause cell death by virtue of releasing nitric oxide, is highly unstable in air and is rapidly oxidized to nitrocobalamin (NO2Cbl). We demonstrate that CblC catalyzes the GSH-dependent denitration of NO2Cbl forming 5-coordinate cob(II)alamin, which had one of two fates. It could be oxidized to aquo-cob(III)alamin or enter a futile thiol oxidase cycle forming GSH disulfide. Arg-161 in the active site of CblC suppressed the NO2Cbl-dependent thiol oxidase activity, whereas the disease-associated R161G variant stabilized cob(II)alamin and promoted futile cycling. We also report that CblC exhibits nitrite reductase activity, converting cob(I)alamin and nitrite to NOCbl. Finally, the denitration activity of CblC supported cell proliferation in the presence of NO2Cbl, which can serve as a cobalamin source. The newly described nitrite reductase and denitration activities of CblC extend its catalytic versatility, adding to its known decyanation and dealkylation activities. In summary, upon exposure to air, NOCbl is rapidly converted to NO2Cbl, which is a substrate for the B12 trafficking enzyme CblC.

Liver Neoplasms

2014 ◽  
Vol 19 (6) ◽  
pp. 277-278
Author(s):  
Matthew M. Yeh
Keyword(s):  
Liver Neoplasms

Regional Arterial Therapy in the Management of Primary Liver Neoplasms

Liver Cancer ◽  
1985 ◽  
pp. 263-273 ◽  
Author(s):  
Yehuda Z. Patt ◽  
Chusilp Charnsangavej ◽  
Marilyn Soski ◽  
Giora M. Mavligit
Keyword(s):  
Liver Neoplasms

Nonepithelial Liver Neoplasms

2013 ◽  
pp. 209-219
Author(s):  
Anthony W. H. Chan ◽  
Alberto Quaglia ◽  
Beate Haugk ◽  
Alastair Burt
Keyword(s):  
Liver Neoplasms
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