PYCR1 antibody
- Known as:
- PYCR1 (anti-)
- Catalog number:
- orb40596
- Product Quantity:
- 20 ug(Trial size)
- Category:
- -
- Supplier:
- Biorb
- Gene target:
- PYCR1 antibody
Related genes to: PYCR1 antibody
- Gene:
- PYCR1 NIH gene
- Name:
- pyrroline-5-carboxylate reductase 1
- Previous symbol:
- -
- Synonyms:
- P5C
- Chromosome:
- 17q25.3
- Locus Type:
- gene with protein product
- Date approved:
- 1992-11-24
- Date modifiied:
- 2015-09-07
Related products to: PYCR1 antibody
Related articles to: PYCR1 antibody
- Myofibroblasts are the cells responsible for collagen production, leading to tissue fibrosis. Because 20.5% of the total amino acids in collagen are proline, myofibroblasts must acquire a well-developed proline-producing mechanism during their differentiation. However, the detailed mechanism for myofibroblasts to acquire and keep the developed proline biosynthesis machinery remains obscure. Here, we show branched-chain amino acid transaminase 1 (Bcat1) is up-regulated in a substantial subset of Postn-expressing proto-myofibroblast-like fibroblasts, transitional cells en route to fully differentiated myofibroblasts, as well as in myofibroblasts in the fibrotic heart and liver of mice and humans and promotes the proline production. The branched-chain amino acid (BCAA) production by BCAT1 promotes SMAD3 phosphorylation via HDAC5 phosphorylation at Ser488, thereby enhancing SMAD3-dependent transcription of proline biosynthesis-related genes, Aldh18a1, Pycr1, and Eprs, in proto-myofibroblast-like fibroblasts and myofibroblasts. In BCAT1-deficient mice, expression of proline biosynthesis-related genes is significantly attenuated in their hearts after myocardial infarction, resulting in decreased cardiac fibrosis. Moreover, BCAT1 inhibitor treatment of mice with myocardial infarction reduces cardiac fibrosis. Our results identified a BCAT1-mediated pathway that promotes collagen production via proline biosynthesis regulation in proto-myofibroblast-like fibroblasts and myofibroblasts, which may provide a therapeutic target for cardiac fibrosis. - Source: PubMed
Publication date: 2026/07/16
Takizawa NoburoHironaka TakanoriWatanabe HayatoSuetsugu HarunaYoshioka KeisukeHorii YumaNagata YuriMatoba HiroakiKosako HidetakaHamase KenjiHirai GoNakaya Michio - PYCR1-related cutis laxa (autosomal recessive cutis laxa type IIB) is a rare inherited connective tissue disorder characterized by loose, inelastic skin, and variable multisystem involvement. While systemic and neurological manifestations have been well described in the literature, data on renal and urinary tract findings remain sparse. We report a 6-year-old girl with genetically confirmed PYCR1-related cutis laxa who presented with recurrent febrile urinary tract infections. Workup revealed bilateral grade V vesicoureteral reflux alongside a small, atrophic left kidney with cortical defects on dimercaptosuccinic acid scintigraphy, in keeping with established reflux nephropathy. Differential renal function was markedly asymmetric, with the left kidney contributing 24% and the right kidney 76% of total function. Despite the degree of unilateral parenchymal loss, estimated glomerular filtration rate and blood pressure remained within age-appropriate limits, though mild proteinuria was noted on further evaluation. Given the burden of recurrent febrile infections in the setting of high-grade bilateral reflux, she was initially managed with prophylactic nitrofurantoin. Following urology consultation surgical correction was therefore pursued, and she underwent bilateral antireflux surgery. She remained free of breakthrough urinary tract infections throughout early postoperative follow-up. The concurrence of severe vesicoureteral reflux, reflux nephropathy, and PYCR1-related cutis laxa has rarely been documented. Whether this association reflects a pathophysiological link or represents coincidental findings in a single patient is not yet clear; nonetheless, this case highlights the importance of considering underlying urological pathology in children with complex connective tissue disorders who present with recurrent urinary tract infections. - Source: PubMed
Publication date: 2026/07/28
Derakhshan DornaBashir FaizanDerakhshan Ali - Hepatocellular carcinoma (HCC) is the most common primary liver cancer in adults, with increasing incidence. It is helpful to establish a prognostic risk prediction model related to macrophage polarization and mitochondrial dysfunction associated with HCC. - Source: PubMed
Publication date: 2026/07/17
Cheng Bin-BinTu Wei-PingTu Xiao-YuLi Bai - Proline metabolism has been associated with schizophrenia pathophysiology; however, the underlying molecular mechanisms remain elusive. This study aimed to investigate the changes in the entire proline metabolic pathway in postmortem brains of patients with schizophrenia. Herein, enzyme-linked immunosorbent assay was performed to determine the protein levels of proline metabolism-associated key enzymes (prolidase [PEPD], proline dehydrogenase [PRODH], pyrroline-5-carboxylate synthetase [ALDH18A1], ornithine aminotransferase [OAT], and pyrroline-5-carboxylate reductase 1 [PYCR1]). Additionally, amino acids metabolized through the proline pathway, including proline, ornithine, and glutamic acid, were quantitatively analyzed through liquid chromatography-tandem mass spectrometry. The alterations in the expression of proline metabolism-associated enzymes and associated amino acid levels were analyzed in the postmortem brains of individuals with schizophrenia, and their associations with premortem clinical symptom scores were examined. Notably, in patients with schizophrenia, PRODH and PYCR1 expression significantly decreased in the superior temporal gyrus and prefrontal cortex, respectively, whereas amino acid levels showed no significant differences. Overall, the findings of this study show that dysregulation in proline metabolism may contribute to mitochondrial dysfunction owing to its close association with energy production and redox regulation. These results suggest the underlying pathophysiology of schizophrenia and provide insights for developing novel therapeutic strategies for managing schizophrenia. - Source: PubMed
Publication date: 2026/06/26
Nagaoka AtsukoHino MizukiShishido RisaHatano MasatakaHosogai YutoKano AkimuHamasaki HideomiKakita AkiyoshiTomita HiroakiKunii Yasuto - Microgravity during spaceflight is increasingly associated with cutaneous impairment, yet the structural basis and underlying metabolic mechanisms remain poorly defined. Collagen, the main component of the dermal extracellular matrix, relies on de novo proline synthesis in fibroblasts. Herein, we examined whether simulated microgravity (SMG) affects collagen synthesis by altering the proline biosynthetic pathway. - Source: PubMed
Publication date: 2026/06/25
Wang WenbinPeng XiufenLuo QingSong Guanbin