{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,16]],"date-time":"2026-01-16T17:51:42Z","timestamp":1768585902824,"version":"3.49.0"},"reference-count":140,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2023,1,14]],"date-time":"2023-01-14T00:00:00Z","timestamp":1673654400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Biomedicines"],"abstract":"<jats:p>Since its discovery in 1955, the understanding of the lysosome has continuously increased. Once considered a mere waste removal system, the lysosome is now recognised as a highly crucial cellular component for signalling and energy metabolism. This notable evolution raises the need for a summarized review of the lysosome\u2019s biology. As such, throughout this article, we will be compiling the current knowledge regarding the lysosome\u2019s biogenesis and functions. The comprehension of this organelle\u2019s inner mechanisms is crucial to perceive how its impairment can give rise to lysosomal disease (LD). In this review, we highlight some examples of LD fine-tuned mechanisms that are already established, as well as others, which are still under investigation. Even though the understanding of the lysosome and its pathologies has expanded through the years, some of its intrinsic molecular aspects remain unknown. In order to illustrate the complexity of the lysosomal diseases we provide a few examples that have challenged the established single gene\u2014single genetic disorder model. As such, we believe there is a strong need for further investigation of the exact abnormalities in the pathological pathways in lysosomal disease.<\/jats:p>","DOI":"10.3390\/biomedicines11010213","type":"journal-article","created":{"date-parts":[[2023,1,16]],"date-time":"2023-01-16T03:10:34Z","timestamp":1673838634000},"page":"213","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":24,"title":["The Biology of Lysosomes: From Order to Disorder"],"prefix":"10.3390","volume":"11","author":[{"given":"Olga","family":"Amaral","sequence":"first","affiliation":[{"name":"Departamento de Gen\u00e9tica Humana, Unidade de Investiga\u00e7\u00e3o e Desenvolvimento, Instituto Nacional de Sa\u00fade Ricardo Jorge (INSA), 4000-055 Porto, Portugal"},{"name":"Centro de Estudos de Ci\u00eancia Animal (CECA, ICETA), Universidade do Porto, 4485-661 Porto, Portugal"},{"name":"Laborat\u00f3rio Associado para Ci\u00eancia Animal e Veterin\u00e1ria (AL4AnimalS), 1300-477 Lisboa, Portugal"}]},{"given":"Mariana","family":"Martins","sequence":"additional","affiliation":[{"name":"Departamento de Ci\u00eancias M\u00e9dicas, Universidade de Aveiro, Campus Universit\u00e1rio de Santiago, Agra do Crasto, Edif\u00edcio 30, 3810-193 Aveiro, Portugal"}]},{"given":"Ana Rita","family":"Oliveira","sequence":"additional","affiliation":[{"name":"Departamento de Ci\u00eancias M\u00e9dicas, Universidade de Aveiro, Campus Universit\u00e1rio de Santiago, Agra do Crasto, Edif\u00edcio 30, 3810-193 Aveiro, Portugal"}]},{"given":"Ana Joana","family":"Duarte","sequence":"additional","affiliation":[{"name":"Departamento de Gen\u00e9tica Humana, Unidade de Investiga\u00e7\u00e3o e Desenvolvimento, Instituto Nacional de Sa\u00fade Ricardo Jorge (INSA), 4000-055 Porto, Portugal"},{"name":"Centro de Estudos de Ci\u00eancia Animal (CECA, ICETA), Universidade do Porto, 4485-661 Porto, Portugal"},{"name":"Laborat\u00f3rio Associado para Ci\u00eancia Animal e Veterin\u00e1ria (AL4AnimalS), 1300-477 Lisboa, Portugal"},{"name":"Instituto de Ci\u00eancias Biom\u00e9dicas Abel Salazar (ICBAS), Universidade do Porto, 4050-313 Porto, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1425-1498","authenticated-orcid":false,"given":"In\u00eas","family":"Mondrag\u00e3o-Rodrigues","sequence":"additional","affiliation":[{"name":"Departamento de Ci\u00eancias M\u00e9dicas, Universidade de Aveiro, Campus Universit\u00e1rio de Santiago, Agra do Crasto, Edif\u00edcio 30, 3810-193 Aveiro, Portugal"},{"name":"CAGE, Instituto de Investiga\u00e7\u00e3o e Inova\u00e7\u00e3o em Sa\u00fade (i3S), Universidade do Porto, Rua Alfredo Allen, 208, 4200-135 Porto, Portugal"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-2252-6105","authenticated-orcid":false,"given":"M. F\u00e1tima","family":"Macedo","sequence":"additional","affiliation":[{"name":"Departamento de Ci\u00eancias M\u00e9dicas, Universidade de Aveiro, Campus Universit\u00e1rio de Santiago, Agra do Crasto, Edif\u00edcio 30, 3810-193 Aveiro, Portugal"},{"name":"CAGE, Instituto de Investiga\u00e7\u00e3o e Inova\u00e7\u00e3o em Sa\u00fade (i3S), Universidade do Porto, Rua Alfredo Allen, 208, 4200-135 Porto, Portugal"}]}],"member":"1968","published-online":{"date-parts":[[2023,1,14]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"847","DOI":"10.1038\/ncb0905-847","article-title":"The lysosome turns fifty","volume":"7","year":"2005","journal-title":"Nat. Cell Biol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1146\/annurev-physiol-021014-071649","article-title":"Lysosomal Physiology","volume":"77","author":"Xu","year":"2015","journal-title":"Annu. Rev. 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