Jove
Visualize
Contact Us
JoVE
x logofacebook logolinkedin logoyoutube logo
ABOUT JoVE
OverviewLeadershipBlogJoVE Help Center
AUTHORS
Publishing ProcessEditorial BoardScope & PoliciesPeer ReviewFAQSubmit
LIBRARIANS
TestimonialsSubscriptionsAccessResourcesLibrary Advisory BoardFAQ
RESEARCH
JoVE JournalMethods CollectionsJoVE Encyclopedia of ExperimentsArchive
EDUCATION
JoVE CoreJoVE BusinessJoVE Science EducationJoVE Lab ManualFaculty Resource CenterFaculty Site
Terms & Conditions of Use
Privacy Policy
Policies

Related Concept Videos

Pyruvate Oxidation01:15

Pyruvate Oxidation

After glycolysis, the charged pyruvate molecules enter the mitochondria via active transport and undergo three enzymatic reactions. These reactions ensure that pyruvate can enter the next metabolic pathway so that energy stored in the pyruvate molecules can be harnessed by the cells.
First, the enzyme pyruvate dehydrogenase removes the carboxyl group from pyruvate and releases it as carbon dioxide. The stripped molecule is then oxidized and releases electrons, which are then picked up by NAD+...
RNA Polymerase II Accessory Proteins02:36

RNA Polymerase II Accessory Proteins

Proteins that regulate transcription can do so either via direct contact with RNA Polymerase or through indirect interactions facilitated by adaptors, mediators, histone-modifying proteins, and nucleosome remodelers. Direct interactions to activate transcription is seen in bacteria as well as in some eukaryotic genes. In these cases, upstream activation sequences are adjacent to the promoters, and the activator proteins interact directly with the transcriptional machinery. For example, in...
Cell Specific Gene Expression01:58

Cell Specific Gene Expression

Multicellular organisms contain a variety of structurally and functionally distinct cell types, but the DNA in all the cells originated from the same parent cells. The differences in the cells can be attributed to the differential gene expression. Liver cells, whose functions include detoxification of blood, production of bile to metabolize fats, and synthesis of proteins essential for metabolism, must express a specific set of genes to perform their functions. Gene expression also varies with...
The ADP/ATP Carrier Protein01:42

The ADP/ATP Carrier Protein

ADP/ATP carrier or AAC protein is the most abundant carrier protein in the inner mitochondrial membrane. It transports large quantities of ADP and ATP, equivalent to the average human body weight, every day. Among other transporters, ACC protein is one of the best-studied members of the mitochondrial carrier protein family. The ADP/ATP carrier protein comprises two transmembrane helices connected to a loop and a single alpha-helix on the matrix side. It switches between two conformational...
Allosteric Proteins-ATCase01:19

Allosteric Proteins-ATCase

Binding sites linkages can regulate a protein's function.  For example, enzyme activity is often regulated through a feedback mechanism where the end product of the biochemical process serves as an inhibitor.
Aspartate transcarbamoylase (ATCase) is a cytosolic enzyme that catalyzes the condensation of L-aspartate and carbamoyl phosphate to  N-carbamoyl-L-aspartate. This reaction is the first step in pyrimidine biosynthesis. UTP and CTP, the end products of the pyrimidine synthesis pathway,...
Prokaryotic Gene Structure and Organization01:28

Prokaryotic Gene Structure and Organization

Prokaryotic genomes exhibit a streamlined organization of coding and non-coding regions essential for gene expression and protein synthesis. While coding regions contain the genetic instructions for proteins or functional RNAs, non-coding regions regulate the precise transcription and translation of these genes.Coding Regions: Proteins and RNAsThe primary coding regions, known as structural genes, include sequences transcribed into messenger RNA (mRNA) and ultimately translated into...

You might also read

Related Articles

Articles linked to this work by shared authors, journal, and citation graph.

Sort by
Same author

Machine learning reveals country-specific drivers of global cancer outcomes.

Annals of oncology : official journal of the European Society for Medical Oncology·2026
Same author

Data driven identification and classification of Chinese medicine syndrome types among functional dyspepsia patients: latent tree analysis (abridged secondary publication).

Hong Kong medical journal = Xianggang yi xue za zhi·2024
Same author

Stability Analysis and Identification of Superior Hybrids in Pearl Millet [<i>Pennisetum glaucum</i> (L.) R. Br.] Using the Multi Trait Stability Index.

Plants (Basel, Switzerland)·2024
Same author

Genetic polymorphism in <i>Methylenetetrahydrofolate Reductase chloride transport protein 6</i> (<i>MTHFR CLCN6</i>) gene is associated with keratinocyte skin cancer in a cohort of renal transplant recipients.

Skin health and disease·2022
Same author

Long-term outcomes of ablation, liver resection, and liver transplant as first-line treatment for solitary HCC of 3 cm or less using an intention-to-treat analysis: A retrospective cohort study.

Annals of medicine and surgery (2012)·2022
Same author

The effects of cesium lead bromide quantum dots on the performance of copper phthalocyanine-based organic field-effect transistors.

Nanotechnology·2021

Related Experiment Video

Updated: Jul 12, 2026

Expression Analysis of Mammalian Linker-histone Subtypes
14:40

Expression Analysis of Mammalian Linker-histone Subtypes

Published on: March 19, 2012

Characterization of cDNAs encoding human pyruvate dehydrogenase alpha subunit.

L Ho1, I D Wexler, T C Liu

  • 1Department of Biochemistry, Case Western Reserve University School of Medicine, Cleveland, OH 44106.

Proceedings of the National Academy of Sciences of the United States of America
|July 1, 1989
PubMed
Summary

Researchers isolated and sequenced the full coding region of human pyruvate dehydrogenase (E1 alpha) precursor. This provides a definitive reference sequence for identifying genetic mutations in related metabolic disorders.

More Related Videos

Expression and Purification of Nuclease-Free Oxygen Scavenger Protocatechuate 3,4-Dioxygenase
10:14

Expression and Purification of Nuclease-Free Oxygen Scavenger Protocatechuate 3,4-Dioxygenase

Published on: November 8, 2019

CD Spectroscopy to Study DNA-Protein Interactions
06:48

CD Spectroscopy to Study DNA-Protein Interactions

Published on: February 10, 2022

Related Experiment Videos

Last Updated: Jul 12, 2026

Expression Analysis of Mammalian Linker-histone Subtypes
14:40

Expression Analysis of Mammalian Linker-histone Subtypes

Published on: March 19, 2012

Expression and Purification of Nuclease-Free Oxygen Scavenger Protocatechuate 3,4-Dioxygenase
10:14

Expression and Purification of Nuclease-Free Oxygen Scavenger Protocatechuate 3,4-Dioxygenase

Published on: November 8, 2019

CD Spectroscopy to Study DNA-Protein Interactions
06:48

CD Spectroscopy to Study DNA-Protein Interactions

Published on: February 10, 2022

Area of Science:

  • Molecular Biology
  • Human Genetics
  • Biochemistry

Background:

  • Pyruvate dehydrogenase (E1 alpha) deficiency is a severe genetic disorder.
  • Accurate genetic sequencing is crucial for diagnosing and understanding E1 alpha deficiency.

Purpose of the Study:

  • To isolate and characterize the complete coding sequence of the human pyruvate dehydrogenase alpha subunit (E1 alpha) precursor.
  • To establish a definitive reference nucleotide sequence for human E1 alpha cDNA.

Main Methods:

  • Isolation of a human liver cDNA library using phage lambda gt11.
  • Nucleotide sequencing of the cDNA clone and confirmation via reverse transcription and polymerase chain reaction (PCR) amplification of RNA fragments.
  • Analysis of the deduced amino acid sequence for mitochondrial targeting signals.

Main Results:

  • A 1423 base pair cDNA clone encoding the entire precursor E1 alpha subunit was obtained.
  • The sequence includes a 29-amino acid mitochondrial targeting leader and a 361-amino acid mature peptide.
  • The 5' and 3' untranslated regions were characterized, including a polyadenylylation signal.

Conclusions:

  • The consensus nucleotide sequence resolves previous discrepancies in human E1 alpha cDNA sequences.
  • This unambiguous sequence is essential for characterizing genetic mutations in pyruvate dehydrogenase deficiency.
  • Facilitates genetic diagnostics and research into E1 alpha-related metabolic diseases.