The Biology of Lysosomes: From Order to Disorder
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...
| Main Authors: | , , , , , |
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| Format: | Article |
| Language: | English |
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MDPI AG
2023-01-01
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| Series: | Biomedicines |
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| Online Access: | https://www.mdpi.com/2227-9059/11/1/213 |
| _version_ | 1827628180324745216 |
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| author | Olga Amaral Mariana Martins Ana Rita Oliveira Ana Joana Duarte Inês Mondragão-Rodrigues M. Fátima Macedo |
| author_facet | Olga Amaral Mariana Martins Ana Rita Oliveira Ana Joana Duarte Inês Mondragão-Rodrigues M. Fátima Macedo |
| author_sort | Olga Amaral |
| collection | DOAJ |
| description | 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’s biology. As such, throughout this article, we will be compiling the current knowledge regarding the lysosome’s biogenesis and functions. The comprehension of this organelle’s 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—single 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. |
| first_indexed | 2024-03-09T13:28:05Z |
| format | Article |
| id | doaj.art-40d35628f24d401b826a8b4a844084ff |
| institution | Directory Open Access Journal |
| issn | 2227-9059 |
| language | English |
| last_indexed | 2024-03-09T13:28:05Z |
| publishDate | 2023-01-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Biomedicines |
| spelling | doaj.art-40d35628f24d401b826a8b4a844084ff2023-11-30T21:21:07ZengMDPI AGBiomedicines2227-90592023-01-0111121310.3390/biomedicines11010213The Biology of Lysosomes: From Order to DisorderOlga Amaral0Mariana Martins1Ana Rita Oliveira2Ana Joana Duarte3Inês Mondragão-Rodrigues4M. Fátima Macedo5Departamento de Genética Humana, Unidade de Investigação e Desenvolvimento, Instituto Nacional de Saúde Ricardo Jorge (INSA), 4000-055 Porto, PortugalDepartamento de Ciências Médicas, Universidade de Aveiro, Campus Universitário de Santiago, Agra do Crasto, Edifício 30, 3810-193 Aveiro, PortugalDepartamento de Ciências Médicas, Universidade de Aveiro, Campus Universitário de Santiago, Agra do Crasto, Edifício 30, 3810-193 Aveiro, PortugalDepartamento de Genética Humana, Unidade de Investigação e Desenvolvimento, Instituto Nacional de Saúde Ricardo Jorge (INSA), 4000-055 Porto, PortugalDepartamento de Ciências Médicas, Universidade de Aveiro, Campus Universitário de Santiago, Agra do Crasto, Edifício 30, 3810-193 Aveiro, PortugalDepartamento de Ciências Médicas, Universidade de Aveiro, Campus Universitário de Santiago, Agra do Crasto, Edifício 30, 3810-193 Aveiro, PortugalSince 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’s biology. As such, throughout this article, we will be compiling the current knowledge regarding the lysosome’s biogenesis and functions. The comprehension of this organelle’s 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—single 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.https://www.mdpi.com/2227-9059/11/1/213lysosomeendocytic pathwaylysosome biogenesis and functionlysosomal disease |
| spellingShingle | Olga Amaral Mariana Martins Ana Rita Oliveira Ana Joana Duarte Inês Mondragão-Rodrigues M. Fátima Macedo The Biology of Lysosomes: From Order to Disorder Biomedicines lysosome endocytic pathway lysosome biogenesis and function lysosomal disease |
| title | The Biology of Lysosomes: From Order to Disorder |
| title_full | The Biology of Lysosomes: From Order to Disorder |
| title_fullStr | The Biology of Lysosomes: From Order to Disorder |
| title_full_unstemmed | The Biology of Lysosomes: From Order to Disorder |
| title_short | The Biology of Lysosomes: From Order to Disorder |
| title_sort | biology of lysosomes from order to disorder |
| topic | lysosome endocytic pathway lysosome biogenesis and function lysosomal disease |
| url | https://www.mdpi.com/2227-9059/11/1/213 |
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