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Related Concept Videos

Membrane Transporters01:31

Membrane Transporters

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Transporters are essential membrane transport proteins with functions related to cell nutrition, homeostasis, communication, etc. Approximately 7% of all genes in the human genome code for transporters or transporter-related proteins.
Transporters are mainly composed of alpha-helices, built from bundles of ten or more helices traversing the plasma membrane. The solute-binding sites are located midway, where some of the helices are broken or distorted, making space for the binding site through...
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The Significance of Membrane Transport01:44

The Significance of Membrane Transport

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The transport of solutes across the cell membrane is essential for metabolic processes, like maintaining cell size and volume, generating the action potential, exchanging nutrients and gases, etc. Membrane transport can be either passive or active. It can be simple diffusion, facilitated, or mediated transport aided by transport proteins such as transporters and channels.
Transporters facilitate either an active or passive movement of solutes. They can allow a single-molecule transport down its...
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Cellular Membranes and Drug Transport01:24

Cellular Membranes and Drug Transport

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Drugs must traverse multiple biological barriers, such as multi-layered skin, single-layered intestinal epithelium, and the plasma membrane, to reach their target sites within the body. The plasma membrane, a highly structured composite of phospholipids, carbohydrates, and proteins, is the cell's protective boundary, facilitating selective substance exchange.
Phospholipids arrange themselves into a bilayer, with hydrophilic heads oriented outward and hydrophobic tails facing inward.
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Membrane Asymmetry Regulating Transporters01:19

Membrane Asymmetry Regulating Transporters

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Enzymes like flippase, floppase, and scramblase transfer phospholipids from one layer to another in the membrane, thereby affecting membrane asymmetry.
Flippase
Eukaryotic flippases are type-IV P-type ATPases or P4-ATPases belonging to P-type ATPase family proteins that are membrane-bound pumps involved in the ATP-mediated transport of ions and molecules across the membrane. Flippases flip specific phospholipids from the outer to the inner leaflet of a membrane. All P4-ATPases have one...
6.9K
Protein Transport to the Inner Chloroplast Membrane01:18

Protein Transport to the Inner Chloroplast Membrane

2.4K
Proteins targeted to the inner chloroplast membrane, or plastid proteins, are transported by two general pathways: the stop-transfer and the re-insertion or post-import pathways. Most plastid proteins carry N-terminal transit sequences and internal import sequences targeting it to the specific chloroplast subcompartment. Proteins targeted by the stop-transfer pathway have internal hydrophobic sequences that inhibit their translocation into the stroma. As a result, these precursors are arrested...
2.4K
Protein Transport into the Inner Mitochondrial Membrane01:34

Protein Transport into the Inner Mitochondrial Membrane

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Nuclear encoded mitochondrial precursors are imported to the inner membrane in a multistep process involving two separate translocons, TIM22 and TIM23. TIM23 is a cation-selective pore that remains closed by the N terminal segment of the protein. Negative charges on the TIM23 act as a receptor for the incoming precursor, pulling the positively charged matrix-targeting sequence for peptide insertion and translocation.
Transport of mitochondrial precursors across the TIM23 channel is driven by...
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Related Experiment Video

Updated: Jan 21, 2026

Characterization of Membrane Transporters by Heterologous Expression in E. coli and Production of Membrane Vesicles
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Characterization of Membrane Transporters by Heterologous Expression in E. coli and Production of Membrane Vesicles

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CDG due to Defective Membrane Transporters: Update.

D Quelhas1,2,3, C R Ferreira4, J Jaeken5

  • 1Unidade de Bioquímica Genética, Serviço de Genética Laboratorial, Centro de Genética Médica, Clínica de Genética e Patologia, Centro Hospitalar Universitário de Santo António, Unidade Local de Saúde de Santo António, Porto, Portugal.

Journal of Inherited Metabolic Disease
|January 19, 2026
PubMed
Summary
This summary is machine-generated.

Congenital disorders of glycosylation (CDG) involve genetic defects in glycan assembly. This review focuses on CDG caused by transporter defects, updating clinical, genetic, and therapeutic knowledge.

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Membrane Transport Processes Analyzed by a Highly Parallel Nanopore Chip System at Single Protein Resolution
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Area of Science:

  • Biochemistry
  • Genetics
  • Cell Biology

Background:

  • Congenital disorders of glycosylation (CDG) are inherited conditions affecting glycan synthesis.
  • Over 200 CDG types are known, primarily enzymatic deficiencies.
  • A subset of CDG involves defects in ER, Golgi apparatus, and plasma membrane transporters.

Purpose of the Study:

  • To provide an updated overview of CDG related to transporter defects.
  • To cover clinical, biochemical, genetic, and therapeutic aspects.
  • To include information on relevant animal models.

Main Methods:

  • Literature review focusing on CDG involving transporters.
  • Exclusion of defects in other cellular trafficking mechanisms.
  • Synthesis of current knowledge on clinical presentation, genetics, and treatment.

Main Results:

  • 13 CDG types (6.5%) are linked to transporter defects.
  • Review details the clinical spectrum, genetic basis, and biochemical findings.
  • Therapeutic strategies and animal models are discussed.

Conclusions:

  • Transporter defects represent a significant, though less common, category of CDG.
  • Comprehensive understanding of these CDG is crucial for diagnosis and management.
  • Further research into therapeutic interventions is warranted.