Related Experiment Video

Updated: Nov 19, 2025

Chemically-blocked Antibody Microarray for Multiplexed High-throughput Profiling of Specific Protein Glycosylation in Complex Samples
13:21

Chemically-blocked Antibody Microarray for Multiplexed High-throughput Profiling of Specific Protein Glycosylation in Complex Samples

Published on: May 4, 2012

16.0K

Pediatric Liver Disease Patients and Secondary Glycosylation Abnormalities

Anna Bogdańska1, Patryk Lipiński2, Paulina Szymańska-Rożek3

  • 1Department of Biochemistry, Radioimmunology and Experimental Medicine, The Children's Memorial Health Institute, Warsaw, Poland.

Frontiers in Pediatrics
|February 1, 2021
PubMed

Insights

Pediatric liver disease is linked to abnormal serum transferrin (Tf) glycosylation, specifically increased asialo- and monosialo-Tf isoforms. This finding aids in diagnosing liver conditions in children.

Area of Science:

  • Biochemistry
  • Pediatric Gastroenterology
  • Clinical Chemistry

Background:

  • Isoelectric focusing (IEF) of serum transferrin (Tf) is the gold standard for diagnosing congenital disorders of glycosylation (CDG).
  • Abnormal Tf glycosylation is documented in adults with liver disease, but not previously characterized in pediatric liver disease.
  • This study investigates Tf glycosylation patterns in children with primary liver conditions.

Purpose of the Study:

  • To characterize serum transferrin (Tf) glycosylation disturbances in pediatric patients with primary liver disease.
  • To establish reference profiles for Tf isoforms in children with acute liver injury/failure (ALI/ALF) and chronic liver disease (CLD).
  • To correlate Tf glycosylation changes with liver disease severity and treatment response.

Main Methods:

  • Serum transferrin (Tf) isoelectric focusing (IEF) analyses were performed on 19 pediatric patients with primary liver diseases between 1995 and 2019.
  • Patients included those with acute liver injury/failure (ALI/ALF), chronic liver disease (CLD), or those evaluated for/undergoing liver transplantation (LTx).
  • Follow-up analyses were conducted to monitor Tf IEF profiles alongside liver function tests.

Main Results:

  • All pediatric patients with primary liver disease exhibited increased asialo-Tf and monosialo-Tf isoforms.
  • Ten out of 12 patients with ALI/ALF showed elevated asialo-, monosialo-, and disialo-Tf.
  • Serum Tf IEF profiles normalized with liver function improvement, whether spontaneous, treatment-induced, or post-liver transplantation.

Conclusions:

  • Pediatric primary liver disease is consistently associated with elevated asialo-Tf and monosialo-Tf isoforms.
  • The absence of elevated trisialo-Tf isoforms is a key differentiator in this pediatric cohort.
  • Serum Tf IEF analysis is a valuable tool for monitoring liver disease activity and recovery in children.

Keywords:
acute liver failure (ALF)acute liver injury (ALI)autoimmune hepatitis (AIH)chronic liver disease (CLD)congenital disorder of glycosylation (CDG)liver transplantationserum transferrin isoelectrofocusing

More Related Videos

Glycan Node Analysis: A Bottom-up Approach to Glycomics11:36

Glycan Node Analysis: A Bottom-up Approach to Glycomics

Published on: May 22, 2016

10.9K
Human Liver Spheroids from Peripheral Blood for Liver Disease Studies09:51

Human Liver Spheroids from Peripheral Blood for Liver Disease Studies

Published on: January 27, 2023

2.0K

Related Concept Videos

Inborn Errors of Metabolism01:20

Inborn Errors of Metabolism

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...
526
Proteoglycans01:05

Proteoglycans

Glycans, a class of complex heterogeneous molecules, can be covalently attached to proteins to form glycosylated proteins that regulate various physiological and pathological processes. Glycosylated proteins or glycoproteins comprise N-linked and O-linked oligosaccharides. O-glycosylation is the most common type of protein glycosylation. Here, glycans attach to the oxygen atom of the hydroxyl groups of Serine or Threonine residues. O-linked glycosylation occurs later in protein processing,...
4.3K
Pharmacokinetics in Pediatric Patients: Drug Metabolism01:24

Pharmacokinetics in Pediatric Patients: Drug Metabolism

In pediatric care, understanding the nuances of hepatic drug metabolism is crucial, as it significantly differs from that of adults. This divergence is primarily due to the developmental stage of drug-metabolizing enzymes, which affects how medications are processed in the body. In neonates, for instance, the activity of Phase I enzymes—critical for the initial breakdown of drugs—is markedly reduced, functioning at just 20–40% of the levels seen in adults. This reduction poses...
89
Glucose Transporters01:27

Glucose Transporters

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:
26.6K
Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test01:22

Effect of Hepatic Disease on Pharmacokinetics: Pathophysiologic Assessment and Liver Function Test

In clinical practice, the direct measurement of hepatic blood flow to evaluate liver function presents significant challenges due to the intricate and specialized nature of the necessary techniques. Consequently, healthcare professionals often rely on empirical estimates derived from thorough patient examinations and liver function tests to gauge liver health. Among the tools at their disposal, the Child–Pugh and MELD scoring systems stand out for their ability to categorize and assess...
79
Protein Glycosylation01:25

Protein Glycosylation

Glycosylation, the most common post-translational modification for proteins, serves diverse functions. Adding sugars to proteins makes the proteins more resistant to proteolytic digestion. Glycosylated proteins can act as markers and receptors to promote cell-cell adhesion. Additionally, they have many essential quality control functions in the cell, such as correct protein folding and facilitating transport of misfolded proteins to the cytosol, which can be degraded.
Glycosylation occurs in...
8.5K