About
2-methylbutyrylglycinuria (2MBG) is an inherited condition in which the body is unable to break down certain proteins, which can lead to a harmful amount of organic acids and toxins in the body. Signs of 2MBG often begin at infancy, sometimes as early as a few days after birth. In other cases, signs do not develop until childhood. Signs of 2MBG include poor appetite, sleeping longer or more often, tiredness, irritability, fever, vomiting, weak muscle tone, delayed growth, tight muscles, and developmental delays. Many of these signs may occur when a baby eats foods that the body cannot break down.
Condition Type:
Secondary
Frequency:
2-methylbutyrylglycinuria (2MBG) is very rare in the United States. This condition is estimated to occur in every 1 out of 250 to 500 people of Hmong ancestry.
More Information for Parents:
Also known as:
- 2MBG
- 2-methylbutyrylglycinuria
- 2-MBCD deficiency
- 2-MBAD deficiency
- Short/branched chain Acyl-CoA dehydrogenase deficiency (SBCAD)
Core Conditions
- Propionic Acidemia (PROP)
- Methylmalonic Acidemia (Methylmalonyl-CoA Mutase Deficiency) (MUT)
- Methylmalonic Acidemia (Cobalamin Conditions)
- Isovaleric Acidemia (IVA)
- 3-Methylcrotonyl-CoA Carboxylase Deficiency (3-MCC)
- 3-Hydroxy-3-Methylglutaric Aciduria (HMG)
- Holocarboxylase Synthetase Deficiency (MCD)
- Beta-Ketothiolase Deficiency (BKT)
- Glutaric Acidemia, Type I (GA-1)
- Mucopolysaccharidosis, Type II (MPS II)
- Biotinidase Deficiency (BIOT)
- Critical Congenital Heart Disease (CCHD)
- Cystic Fibrosis (CF)
- Classic Galactosemia (GALT)
- Hearing Loss or Varying Hearing Levels
- Severe Combined Immunodeficiency (SCID)
- X-Linked Adrenoleukodystrophy (X-ALD)
- Pompe
- Spinal Muscular Atrophy (SMA)
- Mucopolysaccharidosis, Type I (MPS I)
Secondary
- Citrullinemia, Type II (CIT II)
- Hypermethioninemia (MET)
- Benign Hyperphenylalaninemia (H-PHE)
- Biopterin Defect in Cofactor Biosynthesis (BIOPT-BS)
- Biopterin Defect in Cofactor Regeneration (BIOPT-REG)
- Ornithine Transcarbamylase Deficiency (OTC)
- Carbamoyl Phosphate Synthetase Deficiency (CPS)
- Tyrosinemia, Type II (TYR II)
- Tyrosinemia, Type III (TYR III)