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

Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

296
Phenylketonuria (PKU) is a protein metabolism disorder characterized by high blood levels of the amino acid phenylalanine. This results from a mutation in the gene responsible for phenylalanine hydroxylase, an enzyme that converts phenylalanine into tyrosine. When this enzyme is deficient, phenylalanine builds up in the blood, leading to symptoms such as vomiting, rashes, seizures, growth deficiency, and severe mental retardation. An early diagnosis and a diet restricting phenylalanine intake...
296
Glucose Transporters01:27

Glucose Transporters

25.7K
Glucose transporters facilitate the transport of glucose across the cell membrane. In addition to glucose, some glucose transporters can also aid the movement of other hexoses such as fructose, mannose, and galactose.
Facilitated diffusion-glucose transporters (GLUTs) are encoded by the solute-linked carrier (SLC) family 2, subfamily A gene family, or SLC2A. The 14 GLUT protein members are distributed into three classes:
25.7K
Gastritis-II: Pathophysiology01:17

Gastritis-II: Pathophysiology

678
Gastritis is marked by disruption of the mucosal barrier that usually protects the stomach tissue from digestive juices and manifests in acute and chronic forms.
In acute gastritis, the gastric mucosa becomes swollen and red and undergoes superficial erosion. Superficial ulceration may lead to bleeding.
In chronic gastritis, persistent or repeated insults lead to chronic inflammatory changes and, eventually, thinning or atrophy of the gastric tissue.
Gastritis can stem from various causes, each...
678
Inflammatory Bowel Disease III: Diagnostic Studies and Management I-Nutritional Therapy01:30

Inflammatory Bowel Disease III: Diagnostic Studies and Management I-Nutritional Therapy

449
Various diagnostic tests are employed in the diagnostic process for Inflammatory Bowel Disease (IBD), particularly to differentiate between Crohn's disease and ulcerative colitis.
Diagnostic studies
A colonoscopy is the definitive screening test, distinguishing ulcerative colitis from other colon diseases with similar symptoms. During a colonoscopy test, inflamed mucosa with exudate ulcerations can be observed, and biopsies are taken to determine the histologic characteristics of the...
449
Hypoglycemia and Glucagon01:15

Hypoglycemia and Glucagon

371
Without prolonged fasting, healthy individuals maintain blood glucose levels above 3.5 mM due to a well-adapted neuroendocrine counterregulatory system that effectively prevents acute hypoglycemia, a potentially life-threatening condition. The primary clinical scenarios for hypoglycemia encompass diabetes treatment, inappropriate production of endogenous insulin or insulin-like substances by tumors, and the use of glucose-lowering agents in non-diabetic individuals. Notably, hypoglycemia in the...
371
Overview of Protein Metabolism01:21

Overview of Protein Metabolism

2.2K
Proteins are broken down into amino acids during digestion. Unlike fats and carbohydrates, which are stored for later use, proteins are not. Instead, amino acids are either used to produce ATP through oxidation or contribute to the creation of new proteins for the growth and repair of the body. Any surplus amino acids from the diet are converted into glucose or triglycerides rather than excreted.
Amino acids play various roles in the body once they are absorbed into cells. They are restructured...
2.2K

You might also read

Related Articles

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

Sort by
Same author

State of Health Estimation for Lithium-Ion Batteries via Fused Impedance Feature and V‑Transformer-AGLU Network.

ACS omega·2026
Same author

Prognostic determinants of mortality and clinical outcomes in sepsis: a retrospective cohort study.

BMC infectious diseases·2026
Same author

Development and validation of a risk stratification model for sarcopenia in patients with chronic lung disease: a cross-sectional study based on CHARLS data.

BMJ open respiratory research·2026
Same author

The outcomes of isoniazid prophylaxis in LTBI high-risk pediatric patients undergoing hematopoietic stem cell transplantation.

BMC infectious diseases·2026
Same author

Schisanhenol Alleviates Mycophenolic Acid-Induced Intestinal Epithelial Cell Barrier Damage by Activating the Nrf2/HO-1 Signaling Pathway.

Iranian journal of pharmaceutical research : IJPR·2025
Same author

Clinical characteristics and risk factors of tigecycline-induced acute pancreatitis in kidney transplant recipients: a retrospective study.

The Journal of antimicrobial chemotherapy·2025

Related Experiment Video

Updated: Oct 8, 2025

A Model of Chronic Nutrient Infusion in the Rat
08:18

A Model of Chronic Nutrient Infusion in the Rat

Published on: August 14, 2013

12.8K

Glutaric Acidemia, Pathogenesis and Nutritional Therapy.

Qian Li1, Chunlan Yang2, Lijuan Feng2

  • 1Department of Pharmacy, Suizhou Hospital, Hubei University of Medicine, Suizhou, China.

Frontiers in Nutrition
|January 3, 2022
PubMed
Summary

Glutaric acidemia (GA) involves genetic defects in metabolism. Early diagnosis and nutritional therapy are crucial for managing glutaric acidemia type I and type II, preventing neurological damage and other severe symptoms.

Keywords:
Glutaric acidemiagenetic disordersmaintenance therapynutrition therapypathogenesis

More Related Videos

Evaluation of Amino Acid Consumption in Cultured Bone Cells and Isolated Bone Shafts
06:32

Evaluation of Amino Acid Consumption in Cultured Bone Cells and Isolated Bone Shafts

Published on: April 13, 2022

1.9K
Lentiviral Vector-mediated Gene Therapy of Hepatocytes Ex Vivo for Autologous Transplantation in Swine
09:54

Lentiviral Vector-mediated Gene Therapy of Hepatocytes Ex Vivo for Autologous Transplantation in Swine

Published on: November 4, 2018

8.3K

Related Experiment Videos

Last Updated: Oct 8, 2025

A Model of Chronic Nutrient Infusion in the Rat
08:18

A Model of Chronic Nutrient Infusion in the Rat

Published on: August 14, 2013

12.8K
Evaluation of Amino Acid Consumption in Cultured Bone Cells and Isolated Bone Shafts
06:32

Evaluation of Amino Acid Consumption in Cultured Bone Cells and Isolated Bone Shafts

Published on: April 13, 2022

1.9K
Lentiviral Vector-mediated Gene Therapy of Hepatocytes Ex Vivo for Autologous Transplantation in Swine
09:54

Lentiviral Vector-mediated Gene Therapy of Hepatocytes Ex Vivo for Autologous Transplantation in Swine

Published on: November 4, 2018

8.3K

Area of Science:

  • Biochemistry
  • Genetics
  • Metabolic Disorders

Background:

  • Glutaric acidemia (GA) encompasses heterogeneous genetic disorders characterized by deficiencies in amino acid or fatty acid catabolism.
  • GA is classified into type I and type II, based on distinct autosomal recessive mutations affecting critical metabolic genes.

Purpose of the Study:

  • To review the pathogenesis of glutaric acidemia.
  • To discuss current nutritional management strategies for GA, including emergency and long-term therapy.
  • To promote understanding and effective management of GA.

Main Methods:

  • Literature review focusing on pathogenesis and nutritional management of GA.
  • Synthesis of clinical data on GA types I and II.
  • Analysis of nutrition therapy principles in emergency and maintenance settings.

Main Results:

  • GA type I, if not diagnosed early, can lead to irreversible neurological injury.
  • GA type II presents with varied symptoms, including metabolic disturbances and muscle-related issues.
  • Both GA types can be successfully managed with early diagnosis and nutraceutical supplementation.

Conclusions:

  • Early diagnosis is paramount for successful management of glutaric acidemia.
  • Nutritional therapy, encompassing emergency treatment and long-term nutrition, is a cornerstone of GA management.
  • Understanding GA pathogenesis and tailored nutritional interventions are key to improving patient outcomes.