Search research articles

ABOUT JoVE

Overview Leadership Blog JoVE Help Center

AUTHORS

Publishing Process Editorial Board Scope & Policies Peer Review FAQ Submit

LIBRARIANS

Testimonials Subscriptions Access Resources Library Advisory Board FAQ

RESEARCH

JoVE Journal Methods Collections JoVE Encyclopedia of Experiments Archive

EDUCATION

JoVE Core JoVE Business JoVE Science Education JoVE Lab Manual Faculty Resource Center Faculty Site

Terms & Conditions of Use

Related Concept Videos

Multiple Allele Traits

Multiple Allele Traits

The Concept of Multiple Allelism

Protein Complexes with Interchangeable Parts

Protein Complexes with Interchangeable Parts

Groups of proteins may form a complex where each protein in this complex has a different role in the overall execution of the complex’s function. Often some of the proteins in the complex can be replaced by a closely related variant to give a complex that contains many of the same components yet is functionally distinct.
The SCF ubiquitin ligase is a protein complex of five individual proteins. This complex attaches ubiquitin to other target proteins to mark them for degradation. In order to...

Incomplete Dominance

Incomplete Dominance

Gregor Mendel's work (1822 - 1884) was primarily focused on pea plants. Through his initial experiments, he determined that every gene in a diploid cell has two variants called alleles inherited from each parent. He suggested that amongst these two alleles, one allele is dominant in character and the other recessive. The combination of alleles determines the phenotype of a gene in an organism.

Complementation Tests

Complementation Tests

A complementation test is a simple cross to identify whether the two mutations are located on the same gene or different genes. It was first performed by Edward Lewis in the 1940s while working on fruit flies. He developed the test to identify the location and arrangement of different mutations on chromosomes.
Organisms heterozygous for different mutations are crossed pairwise in all combinations. If present on different genes, the mutations can complement each other by providing the missing...

Epistasis Analysis

Epistasis Analysis

Although Mendel chose seven unrelated traits in peas to study gene segregation, most traits involve multiple gene interactions that create a spectrum of phenotypes. When the interaction of various genes or alleles at different locations influences a phenotype, this is called epistasis. Epistasis often involves one gene masking or interfering with the expression of another (antagonistic epistasis). Epistasis often occurs when different genes are part of the same biochemical pathway. The...

Protein Complexes with Interchangeable Parts

Protein Complexes with Interchangeable Parts

Groups of proteins may form a complex where each protein in this complex has a different role in the overall execution of the complex’s function. Often some of the proteins in the complex can be replaced by a closely related variant to give a complex that contains many of the same components yet is functionally distinct.
The SCF ubiquitin ligase is a protein complex of five individual proteins. This complex attaches ubiquitin to other target proteins to mark them for degradation. In order to...

You might also read

Related Articles

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

Sort by

Same author

Low dose intrathecal morphine for post-cesarean analgesia with scheduled multimodal pain regimen: a prospective, randomized, open blinded end-point study.

International journal of obstetric anesthesia·2025

Same author

Letter to the Editor with regards to the article: Biotinidase deficiency in a Newborn.

Journal of neonatal-perinatal medicine·2023

Same author

Advanced technique for measuring relative length changes under control of temperature and helium-gas pressure.

The Review of scientific instruments·2022

Same author

Gender and racial/ethnic differences in physiologic responses in the Stimulant Reduction Intervention using Dosed Exercise Study.

Addictive behaviors·2020

Same author

Preservation of the mesureter to reduce urinary complications: analysis of data from the observational Leipzig School MMR study.

BJOG : an international journal of obstetrics and gynaecology·2020

Same author

Specific Heat Study of 1D and 2D Excitations in the Layered Frustrated Quantum Antiferromagnets Cs_{2}CuCl_{4-x}Br_{x}.

Physical review letters·2019

Search research articles

Related Experiment Video

Updated: May 12, 2026

Functional Complementation Analysis (FCA): A Laboratory Exercise Designed and Implemented to Supplement the Teaching of Biochemical Pathways

Functional Complementation Analysis (FCA): A Laboratory Exercise Designed and Implemented to Supplement the Teaching of Biochemical Pathways

Published on: June 24, 2016

Heterozygote expression in propionyl coenzyme A carboxylase deficiency. Differences between major complementation

B Wolf, L E Rosenberg

The Journal of Clinical Investigation

|November 1, 1978

Summary

Propionyl coenzyme A carboxylase (PCC) deficiency heterozygotes in the PCC C group showed normal enzyme activity, unlike the PCC A group. This suggests compensatory mechanisms in PCC C heterozygotes, not simple gene dosage effects.

More Related Videos

High-resolution Melting PCR for Complement Receptor 1 Length Polymorphism Genotyping: An Innovative Tool for Alzheimer's Disease Gene Susceptibility Assessment

High-resolution Melting PCR for Complement Receptor 1 Length Polymorphism Genotyping: An Innovative Tool for Alzheimer's Disease Gene Susceptibility Assessment

Published on: July 18, 2017

Quantification of Coenzyme A in Cells and Tissues

Quantification of Coenzyme A in Cells and Tissues

Published on: September 27, 2019

Related Experiment Videos

Last Updated: May 12, 2026

Functional Complementation Analysis (FCA): A Laboratory Exercise Designed and Implemented to Supplement the Teaching of Biochemical Pathways

Functional Complementation Analysis (FCA): A Laboratory Exercise Designed and Implemented to Supplement the Teaching of Biochemical Pathways

Published on: June 24, 2016

High-resolution Melting PCR for Complement Receptor 1 Length Polymorphism Genotyping: An Innovative Tool for Alzheimer's Disease Gene Susceptibility Assessment

High-resolution Melting PCR for Complement Receptor 1 Length Polymorphism Genotyping: An Innovative Tool for Alzheimer's Disease Gene Susceptibility Assessment

Published on: July 18, 2017

Quantification of Coenzyme A in Cells and Tissues

Quantification of Coenzyme A in Cells and Tissues

Published on: September 27, 2019

Area of Science:

Biochemistry
Genetics
Metabolic Disorders

Background:

Propionyl coenzyme A carboxylase (PCC) is crucial for amino acid metabolism.
PCC deficiency is an inherited metabolic disorder.
Heterozygotes for PCC deficiency are typically asymptomatic.

Purpose of the Study:

To investigate propionyl coenzyme A carboxylase (PCC) activity in heterozygotes of different complementation groups.
To determine if gene dosage effects explain enzyme activity levels in heterozygotes.
To explore potential compensatory mechanisms in PCC deficiency heterozygotes.

Main Methods:

Enzyme activity assays were performed on skin fibroblast and peripheral blood leukocyte extracts.
PCC activity was measured in controls and obligate heterozygotes from PCC A and PCC C complementation groups.
Activities of other mitochondrial enzymes were assessed for comparison.

Main Results:

PCC A heterozygotes exhibited approximately 52% of control PCC activity in both cell types.
PCC C heterozygotes showed PCC activity indistinguishable from controls.
Activities of beta-methyl-crotonyl CoA carboxylase and glutamate dehydrogenase were similar across all groups.

Conclusions:

Data from PCC A heterozygotes align with expected gene dosage effects.
Results from PCC C heterozygotes suggest mechanisms beyond simple gene dosage.
Unbalanced synthesis/degradation of PCC subunits with compensatory balancing in PCC C heterozygotes is a plausible explanation.