Results 51 to 60 of about 12,596 (155)

Tissue-specific roles of peroxisomes revealed by expression meta-analysis

Biology Direct
Peroxisomes are primarily studied in the brain, kidney, and liver due to the conspicuous tissue-specific pathology of peroxisomal biogenesis disorders. In contrast, little is known about the role of peroxisomes in other tissues such as the heart. In this
Matthias Plessner, Leonie Thiele, Julia Hofhuis, Sven Thoms   +3 more
doaj   +1 more source

PIP4K2A regulates intracellular cholesterol transport through modulating PI(4,5)P2 homeostasis

Journal of Lipid Research, 2018
The transport of LDL-derived cholesterol from lysosomes to peroxisomes is facilitated by membrane contacts formed between the lysosomal protein synaptotagmin VII and the peroxisomal lipid phosphatidylinositol 4, 5-bisphosphate [PI(4,5)P2].
Ao Hu, Xue-Tong Zhao, Heng Tu, Ting Xiao, Ting Fu, Yan Wang, Yong Liu, Xiong-Jie Shi, Jie Luo, Bao-Liang Song   +9 more
doaj   +1 more source

Peroxisomal dysfunction interferes with odontogenesis and leads to developmentally delayed teeth and defects in distinct dental cells in Pex11b-deficient mice.

PLoS ONE
Human peroxisomal biogenesis disorders of the Zellweger syndrome spectrum affect skeletal development and induce tooth malformations. Whereas several peroxisomal knockout mouse studies elucidated the pathogenesis of skeletal defects, little information ...
Claudia Colasante, Julia Jednakowski, Klaus-Peter Valerius, Xiaoling Li, Eveline Baumgart-Vogt   +4 more
doaj   +1 more source

Role and Function of Peroxisomes in Neuroinflammation

Cells
Peroxisomes are organelles involved in many cellular metabolic functions, including the degradation of very-long-chain fatty acids (VLCFAs; C ≥ 22), the initiation of ether-phospholipid synthesis, and the metabolism of reactive oxygen species.
Chinmoy Sarkar, Marta M. Lipinski
doaj   +1 more source

HSD17B4 deficiency causes dysregulation of primary cilia and is alleviated by acetyl-CoA

Nature Communications
Primary cilia are dynamic sensory organelles orchestrating key signaling pathways, and disruption of primary ciliogenesis is implicated in a spectrum of genetic disorders. The peroxisomal bifunctional enzyme HSD17B4 is pivotal for peroxisomal β-oxidation
Ji-Eun Bae, Soyoung Jang, Joon Bum Kim, Na Yeon Park, Doo Sin Jo, Hyejin Hyung, Pansoo Kim, Min-Seon Kim, Hong-Yeoul Ryu, Hyun-Shik Lee, Dong-Seok Lee, Myriam Baes, Zae Young Ryoo, Dong-Hyung Cho   +13 more
doaj   +1 more source

Eyes See what the Mind Knows: Clues to Pattern Recognition in Single Enzyme Deficiency-Related Peroxisomal Disorders. [PDF]

Mol Syndromol, 2020
Arora V, Bijarnia-Mahay S, Dubey S, Saxena R.   +3 more
europepmc   +1 more source

Comparison of the Diagnostic Performance of C26:0-Lysophosphatidylcholine and Very Long-Chain Fatty Acids Analysis for Peroxisomal Disorders. [PDF]

Front Cell Dev Biol, 2020
Jaspers YRJ, Ferdinandusse S, Dijkstra IME, Barendsen RW, van Lenthe H, Kulik W, Engelen M, Goorden SMI, Vaz FM, Kemp S.   +9 more
europepmc   +1 more source

Serum very long-chain fatty acids (VLCFA) levels as predictive biomarkers of diseases severity and probability of survival in peroxisomal disorders. [PDF]

PLoS One, 2020
Stradomska TJ, Syczewska M, Jamroz E, Pleskaczyńska A, Kruczek P, Ciara E, Tylki-Szymanska A.   +6 more
europepmc   +1 more source

A diagnostic and management odyssey of a rare case of rhizomelic chondrodysplasia punctata

Journal of the Pakistan Medical Association
Rhizomelic chondrodysplasia punctata (RCDP) is one of the rare inherited peroxisomal disorders that belongs to the heterogeneous group of chondrodysplasias presenting with proximal shortening of the limbs and distinctive punctate calcifications of bones
Qurat Ul Ain, Alia Halim, Asma Shabbir , Amina Marriam   +3 more
doaj   +1 more source

Chronic Progressive Spastic Paraparesis: Think of Peroxisomal Disorders - A Case Report of X-Linked Adult Onset Adrenoleukodystrophy With an Update on The Latest Treatment Strategies. [PDF]

Cureus, 2020
Kesserwani H.
europepmc   +1 more source