Organelle Diseases

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چکیده

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منابع مشابه

Molecular mechanisms of organelle biogenesis and related metabolic diseases.

Organelle biogenesis is regulated by transcriptional networks that modulate expression of specific genes encoding organellar proteins. Structural and functional specificity of organelles requires not only the transcription of specific genes and translation of resulting mRNAs, but also the transfer of encoded polypeptides to their site of function through signaling peptides. A defect in targetin...

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Organelle proteomics.

This unit describes strategies for studying the proteomes of organelles, which is one example of targeted proteomics. It relies heavily on previously published units dealing with organelle preparation, protein solubilization, and proteomics techniques. A specific commentary for organelle proteomics is provided. Specific protocols for the isolation of nuclei from various sources (cell cultures, ...

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Organelle Inheritance

The need to inherit those membranes that arise from the ER is much less clear. As an example, the integral *Imperial Cancer Research Fund Cell Biology Laboratory membrane proteins and lipids of the Golgi apparatus are derived entirely from the ER. If a daughter did not 44 Lincoln's Inn Fields London WC2A 3PX inherit a copy of the Golgi apparatus, it should be able to synthesize one de novo. Sev...

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An organelle-specific protein landscape identifies novel diseases and molecular mechanisms

Cellular organelles provide opportunities to relate biological mechanisms to disease. Here we use affinity proteomics, genetics and cell biology to interrogate cilia: poorly understood organelles, where defects cause genetic diseases. Two hundred and seventeen tagged human ciliary proteins create a final landscape of 1,319 proteins, 4,905 interactions and 52 complexes. Reverse tagging, repetiti...

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Mechanisms of organelle biogenesis govern stochastic fluctuations in organelle abundance

Fluctuations in organelle abundance can profoundly limit the precision of cell biological processes from secretion to metabolism. We modeled the dynamics of organelle biogenesis and predicted that organelle abundance fluctuations depend strongly on the specific mechanisms that increase or decrease the number of a given organelle. Our model exactly predicts the size of experimentally measured Go...

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ژورنال

عنوان ژورنال: The American Journal of Human Genetics

سال: 1999

ISSN: 0002-9297

DOI: 10.1086/302178