WFS1 pAb host: Rabbit
- Known as:
- WFS1 pAb production species: Rabbit
- Catalog number:
- bs60455
- Product Quantity:
- USD
- Category:
- -
- Supplier:
- Bioworld
- Gene target:
- WFS1 pAb host: Rabbit
Ask about this productRelated genes to: WFS1 pAb host: Rabbit
- Gene:
- PPP1R18 NIH gene
- Name:
- protein phosphatase 1 regulatory subunit 18
- Previous symbol:
- KIAA1949
- Synonyms:
- phostensin
- Chromosome:
- 6p21.33
- Locus Type:
- gene with protein product
- Date approved:
- 2004-03-02
- Date modifiied:
- 2016-10-05
- Gene:
- WFS1 NIH gene
- Name:
- wolframin ER transmembrane glycoprotein
- Previous symbol:
- DFNA6, DFNA14, DFNA38
- Synonyms:
- DIDMOAD, WFS
- Chromosome:
- 4p16.1
- Locus Type:
- gene with protein product
- Date approved:
- 1995-01-30
- Date modifiied:
- 2016-02-17
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- Dominant variants in (wolframin ER transmembrane glycoprotein), the gene coding for a mitochondria-associated endoplasmic reticulum (ER) membrane (MAM) resident protein, have been associated with Wolfram-like syndrome (WLS). and , WFS1 loss results in reduced ER to mitochondria calcium (Ca) transfer, mitochondrial dysfunction, and enhanced macroautophagy/autophagy and mitophagy. However, in the WLS pathological context, whether the mutant protein triggers the same cellular processes is unknown. Here, we show that in human fibroblasts and murine neuronal cultures the WLS protein WFS1 leads to decreases in mitochondria bioenergetics and Ca uptake, deregulation of the mitochondrial quality system mechanisms, and alteration of the autophagic flux. Moreover, in the mouse, these alterations are concomitant with a decrease of MAM number. These findings reveal pathophysiological similarities between WS and WLS, highlighting the importance of WFS1 for MAM's integrity and functionality. It may open new treatment perspectives for patients with WLS. BafA1: bafilomycin A; ER: endoplasmic reticulum; HSPA9/GRP75: heat shock protein family A (Hsp70) member 9; ITPR/IPR: inositol 1,4,5-trisphosphate receptor; MAM: mitochondria-associated endoplasmic reticulum membrane; MCU: mitochondrial calcium uniporter; MFN2: mitofusin 2; OCR: oxygen consumption rate; ROS: reactive oxygen species; ROT/AA: rotenone+antimycin A; VDAC1: voltage dependent anion channel 1; WLS: Wolfram-like syndrome; WS: Wolfram syndrome; WT: wild-type. - Source: PubMed
Publication date: 2024/04/23
Patergnani SimoneBataillard Méghane SDanese AlbertoAlves StacyCazevieille ChantalValéro RenéTranebjærg LisbethMaurice TanguiPinton PaoloDelprat BenjaminRichard Elodie M - Assessing and responding to threats is vital in everyday life. Unfortunately, many mental illnesses involve impaired risk assessment, affecting patients, families, and society. The brain processes behind these behaviors are not well understood. We developed a transgenic mouse model (disrupted-in-schizophrenia 1 [DISC1]-N) with a disrupted avoidance response in risky settings. Our study utilized single-nucleus RNA sequencing and path-clamp coupling with real-time RT-PCR to uncover a previously undescribed group of glutamatergic neurons in the basolateral amygdala (BLA) marked by Wolfram syndrome 1 (WFS1) expression, whose activity is modulated by adjacent astrocytes. These neurons in DISC1-N mice exhibited diminished firing ability and impaired communication with the astrocytes. Remarkably, optogenetic activation of these astrocytes reinstated neuronal excitability via D-serine acting on BLA neurons' NMDA receptors, leading to improved risk-assessment behavior in the DISC1-N mice. Our findings point to BLA astrocytes as a promising target for treating risk-assessment dysfunctions in mental disorders. - Source: PubMed
Publication date: 2024/04/16
Zhou XinyiXiao QianLiu YaohuiChen ShuaiXu XirongZhang ZhigangHong YuchuanShao JieChen YuewenChen YuWang LipingYang FanTu Jie - Papillary thyroid carcinoma (PTC) accounts for the majority of thyroid cancers and has a high recurrence rate. We aimed to screen key genes involved in PTC to provide novel insights into the mechanisms of PTC. - Source: PubMed
Publication date: 2024/03/15
Pan LingfengZhang LianboFu JingyaoShen KeyuZhang Guang - We aimed to verify the usefulness of targeted next-generation sequencing (NGS) technology for diagnosing monogenic diabetes in a single center. - Source: PubMed
Publication date: 2023/11/15
Takase KaoruSusa ShinjiSato HidenoriHada YurikaNagaoka KyokoTakakubo NoeKarasawa ShigeruKameda WataruNumakura ChikahikoIshizawa Kenichi - Hemocytes are important targets for heavy metal-induced immunotoxicity in insects. This study aimed to investigate the mechanism by which cadmium (Cd) exposure affects the hemocyte count in Lymantria dispar larvae. The results showed that the number of larval hemocytes was significantly decreased under Cd exposure, accompanied by a significant increase in the apoptosis rate and the expression of Caspase-3. The endoplasmic reticulum (ER) of hemocytes in the Cd-treated group showed irregular swelling. Expression levels of ER stress indicator genes (CHOP, Bip1, Bip2, Bip3, and Bip4) were significantly higher in the Cd-treated group. Among the three pathways that potentially mediate ER stress, only the key genes in the ATF6 pathway (ATF6, S1P-1, S1P-2, and WFS1) exhibited differential responses to Cd exposure. Cd exposure significantly increased the levels of reactive oxygen species (ROS) and the expression of oxidative stress-related genes (CNCC, P38, and ATF2) in hemocytes. Studies using inhibitors confirmed that apoptosis mediated the decrease in hemocyte count, ER stress mediated apoptosis, ATF6 pathway mediated ER stress, and ROS or oxidative stress mediated ER stress through the activation of the ATF6 pathway. Taken together, the ROS-ATF6-ER stress-apoptosis pathway is responsible for the reduction in the hemocyte count of Cd-treated L. dispar larvae. - Source: PubMed
Publication date: 2024/03/17
Yue FusenXu JinshengMeng LinyiWang QiTan MingtaoZhang AoyingYan ShanchunJiang Dun