Hemoglobin-Advanced Glycosylation End Products (HB-AGE) ELISA Kit for Cat

The gamma globin genes (HBG1 and HBG2) are normally expressed in the fetal liver, spleen and bone marrow. Two gamma chains together with two alpha chains constitute fetal hemoglobin (HbF) which is normally replaced by adult hemoglobin (HbA) at birth. In some beta-thalassemias and related conditions, gamma chain production continues into adulthood. The two types of gamma chains differ at residue 136 where glycine is found in the G-gamma product (HBG2) and alanine is found in the A-gamma product (HBG1). The former is predominant at birth. The order of the genes in the beta-globin cluster is: 5'- epsilon -- gamma-G -- gamma-A -- delta -- beta--3'. [provided by RefSeq, Jul 2008]

HBG2: Gamma chains make up the fetal hemoglobin F, in combination with alpha chains. Defects in HBG2 are the cause of cyanosis transient neonatal (TNCY). TNCY is a disorder characterized by cyanosis in the fetus and neonate, due to a defect in the fetal hemoglobin chain which has reduced affinity for oxygen. Some patients develop anemia resulting from increased destruction of red cells containing abnormal or unstable hemoglobin. The cyanosis resolves spontaneously by 5 to 6 months of age or earlier, as the adult beta-globin chain is produced and replaces the fetal gamma- globin chain. Belongs to the globin family.

Protein type: Carrier

Chromosomal Location of Human Ortholog: 11p15.5

Cellular Component: hemoglobin complex; cytosol

Molecular Function: iron ion binding; heme binding; oxygen binding; oxygen transporter activity

Biological Process: oxygen transport; blood coagulation

Disease: Cyanosis, Transient Neonatal; Fetal Hemoglobin Quantitative Trait Locus 1