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2016 5
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Live imaging of intra-lysosome pH in cell lines and primary neuronal culture using a novel genetically encoded biosensor.
Ponsford AH, Ryan TA, Raimondi A, Cocucci E, Wycislo SA, Fröhlich F, Swan LE, Stagi M. Ponsford AH, et al. Autophagy. 2021 Jun;17(6):1500-1518. doi: 10.1080/15548627.2020.1771858. Epub 2020 Jun 9. Autophagy. 2021. PMID: 32515674 Free PMC article.
Finally, we demonstrated that while overexpression of ARL8B GTPase causes striking alkalinization of peripheral lysosomes in HEK293 T cells, peripheral lysosomes per se are no less acidic than juxtanuclear lysosomes in our cell lines.Abbreviations: ARL8B: ADP ribosylation factor …
Finally, we demonstrated that while overexpression of ARL8B GTPase causes striking alkalinization of peripheral lysosomes in HEK293 T cells, …
Control of Golgi- V-ATPase through Sac1-dependent co-regulation of PI(4)P and cholesterol.
Zhou X, van der Stoel MM, Kaptan S, Li H, Li S, Hölttä M, Vihinen H, Jokitalo E, Thiele C, Pietiläinen O, Morioka S, Sasaki J, Sasaki T, Vattulainen I, Ikonen E. Zhou X, et al. Nat Commun. 2025 Aug 21;16(1):7808. doi: 10.1038/s41467-025-63125-7. Nat Commun. 2025. PMID: 40841558 Free PMC article.
The TGN disintegration results from its acute deacidification caused by disassembly of the Golgi V-ATPase. Mechanistically, Sac1 mediated TGN membrane composition maintains an assembly-promoting conformation of the V(0)a2 subunit. ...Collectively, our finding …
The TGN disintegration results from its acute deacidification caused by disassembly of the Golgi V-ATPase. Mechanistically, Sa …
Golgi pH elevation due to loss of V-ATPase subunit V0a2 function correlates with tissue-specific glycosylation changes and globozoospermia.
Kopp J, Jahn D, Vogt G, Psoma A, Ratto E, Morelle W, Stelzer N, Hausser I, Hoffmann A, de Los Santos MR, Koch LA, Fischer-Zirnsak B, Thiel C, Palm W, Meierhofer D, van den Bogaart G, Foulquier F, Meinhardt A, Kornak U. Kopp J, et al. Cell Mol Life Sci. 2024 Dec 16;82(1):4. doi: 10.1007/s00018-024-05506-7. Cell Mol Life Sci. 2024. PMID: 39680136 Free PMC article.
Loss-of-function variants in ATP6V0A2, encoding the trans Golgi V-ATPase subunit V0a2, cause wrinkly skin syndrome (WSS), a connective tissue disorder with glycosylation defects and aberrant cortical neuron migration. We used knock-out (Atp6v0a2(-/-)) …
Loss-of-function variants in ATP6V0A2, encoding the trans Golgi V-ATPase subunit V0a2, cause wrinkly skin syndrome (WSS …
Two novel homozygous variants of ATP6V0A2 and ALDH18A1 lead to autosomal recessive cutis laxa type 2 and 3 in two Pakistani families.
Zaman Q, Iftikhar A, Rehman G, Khan Q, Najumuddin, Jan A, Khan J, Anas M, Laiba, Umair M, Muthaffar OY, Abdulkareem AA, Bibi F, Naseer MI, Jelani M. Zaman Q, et al. J Gene Med. 2023 Oct;25(10):e3522. doi: 10.1002/jgm.3522. Epub 2023 Apr 29. J Gene Med. 2023. PMID: 37119015
It is caused by biallelic variation in ATPase, H(+) transporting V0 subunit A2 (ATP6V0A2; OMIM: 611716) located on chromosome 12q24.31. Autosomal recessive cutis laxa type 3A (ARCL3A; OMIM: 219150) is another subclinical type characterized by short stature, ophthalm …
It is caused by biallelic variation in ATPase, H(+) transporting V0 subunit A2 (ATP6V0A2; OMIM: 611716) located on chromosome …
Human V-ATPase a-subunit isoforms bind specifically to distinct phosphoinositide phospholipids.
Mitra C, Winkley S, Kane PM. Mitra C, et al. J Biol Chem. 2023 Dec;299(12):105473. doi: 10.1016/j.jbc.2023.105473. Epub 2023 Nov 17. J Biol Chem. 2023. PMID: 37979916 Free PMC article.
Humans have four a-subunit isoforms, and we hypothesize that the aNT domains of these isoforms will also bind to specific PIP lipids. The a1 and a2 isoforms of human V-ATPase a-subunits are localized to endolysosomes and Golgi, respectively. ...
Humans have four a-subunit isoforms, and we hypothesize that the aNT domains of these isoforms will also bind to specific PIP lipids. The a1 …
Neutrophil-derived serine proteases induce FOXA2-mediated autophagy dysfunction and exacerbate colitis-associated carcinogenesis via protease activated receptor 2.
Yuan J, Ma J, Zhang F, Wang T, Jian X, Wang B, Li W, Zhang X, Cao Y, Yang H, Ma Y, Wang H. Yuan J, et al. Autophagy. 2025 Oct;21(10):2130-2147. doi: 10.1080/15548627.2025.2489335. Epub 2025 Apr 17. Autophagy. 2025. PMID: 40205686 Free PMC article.
Here, we show that F2rl1/Par2 deficiency (F2rl1[deltaIEC]) aggravated azoxymethane-dextran sulfate sodium-induced CAC based on tumor number and burden, promoted autophagy dysfunction characterized by SQSTM1/p62 accumulation and autophagosome-lysosome fusion inhibition in IECs, an …
Here, we show that F2rl1/Par2 deficiency (F2rl1[deltaIEC]) aggravated azoxymethane-dextran sulfate sodium-induced CAC based on tumor number …
Molecular mechanisms of cutis laxa- and distal renal tubular acidosis-causing mutations in V-ATPase a subunits, ATP6V0A2 and ATP6V0A4.
Esmail S, Kartner N, Yao Y, Kim JW, Reithmeier RAF, Manolson MF. Esmail S, et al. J Biol Chem. 2018 Feb 23;293(8):2787-2800. doi: 10.1074/jbc.M117.818872. Epub 2018 Jan 8. J Biol Chem. 2018. PMID: 29311258 Free PMC article.
The a subunit is the largest of 15 different subunits that make up the vacuolar H(+)-ATPase (V-ATPase) complex, where it functions in proton translocation. In mammals, this subunit has four paralogous isoforms, a1-a4, which may encode signals for targeting as …
The a subunit is the largest of 15 different subunits that make up the vacuolar H(+)-ATPase (V-ATPase) complex, where i …
The V-ATPase a3 Subunit: Structure, Function and Therapeutic Potential of an Essential Biomolecule in Osteoclastic Bone Resorption.
Chu A, Zirngibl RA, Manolson MF. Chu A, et al. Int J Mol Sci. 2021 Jun 28;22(13):6934. doi: 10.3390/ijms22136934. Int J Mol Sci. 2021. PMID: 34203247 Free PMC article. Review.
This review focuses on one of the 16 proteins composing the V-ATPase complex responsible for resorbing bone: the a3 subunit. The rationale for focusing on this biomolecule is that mutations in this one protein account for over 50% of osteopetrosis cases, highlightin …
This review focuses on one of the 16 proteins composing the V-ATPase complex responsible for resorbing bone: the a3 subunit. T …
Proteomic analyses reveal lower expression of TEX40 and ATP6V0A2 proteins related to calcium ion entry and acrosomal acidification in asthenozoospermic males.
Sinha A, Singh V, Singh S, Yadav S. Sinha A, et al. Life Sci. 2019 Feb 1;218:81-88. doi: 10.1016/j.lfs.2018.12.016. Epub 2018 Dec 11. Life Sci. 2019. PMID: 30550884
KEY FINDINGS: Although seven differentially regulated proteins were identified, highpoints of the study were two proteins, TEX40 and ATP6V0A2. Lower expression of a crucial sperm motility related protein, TEX40 is reported for the first time in clinically diagnosed AS male …
KEY FINDINGS: Although seven differentially regulated proteins were identified, highpoints of the study were two proteins, TEX40 and ATP6 …
PHY34 inhibits autophagy through V-ATPase V0A2 subunit inhibition and CAS/CSE1L nuclear cargo trafficking in high grade serous ovarian cancer.
Salvi A, Young AN, Huntsman AC, Pergande MR, Korkmaz MA, Rathnayake RA, Mize BK, Kinghorn AD, Zhang X, Ratia K, Schirle M, Thomas JR, Brittain SM, Shelton C, Aldrich LN, Cologna SM, Fuchs JR, Burdette JE. Salvi A, et al. Cell Death Dis. 2022 Jan 10;13(1):45. doi: 10.1038/s41419-021-04495-w. Cell Death Dis. 2022. PMID: 35013112 Free PMC article.
Compounds with a diphyllin structure similar to PHY34 have been shown to inhibit the ATP6V0A2 subunit of V(vacuolar)-ATPase. Therefore, ATP6V0A2 wild-type and ATP6V0A2 V823 mutant cell lines were tested with PHY34, and it was able to induce cell death in the …
Compounds with a diphyllin structure similar to PHY34 have been shown to inhibit the ATP6V0A2 subunit of V(vacuolar)-ATPase. Therefor …
45 results