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carboxylase subunit beta is an enzyme that in humans is encoded by the MCCC2 gene. MCCC2 encodes the β-subunit of the mitochondrial enzyme methylcrotonoyl-CoA
MCCC2
Medical condition
known as MMCA, or the MCCC2 gene, formerly known as MCCB. MCCC1 encodes the a-subunits of 3-methylcrotonyl-CoA carboxylase while MCCC2 encodes the b-subunits
3-Methylcrotonyl-CoA carboxylase deficiency
3-Methylcrotonyl-CoA_carboxylase_deficiency
Class of enzymes
and MCCCβ, encoded by MCCC1 and MCCC2 respectively. MCCC1 gene has 21 exons and resides on chromosome 3 at q27. MCCC2 gene has 19 exons and resides on
Methylcrotonyl-CoA carboxylase
Methylcrotonyl-CoA_carboxylase
Human chromosome
susceptibility 1 homolog (mouse) MCC: Colorectal mutant cancer protein MCCC2: methylcrotonoyl-Coenzyme A carboxylase 2 (beta) MCEF: transcription factor
Chromosome_5
HGNC:29622; Q8IVS2 9341 MCC HGNC:6935; P23508 9342 MCCC1 HGNC:6936; Q96RQ3 9343 MCCC2 HGNC:6937; Q9HCC0 9344 MCCD1 HGNC:20668; P59942 9345 MCEE HGNC:16732; Q96PE7
List of human protein-coding genes 5
List_of_human_protein-coding_genes_5
Protein-coding gene in the species Homo sapiens
ECHS1, HADHA, HADHB, ACAA2); Amino acid catabolic pathways proteins (MCCC1, MCCC2, GLUD1, HIBADH, CPS1); Amino acid biosynthesis proteins (PYCR1, PYCR2, ALDH18A1
FASTKD3
Topics referred to by the same term
Clinic Cancer Center, a research institute in the United States MCCC1 and MCCC2, genes that encode methylcrotonyl-CoA carboxylase Midwest Christian College
MCCC
deficiency; 210200; MCCC1 3-Methylcrotonyl-CoA carboxylase 2 deficiency; 210210; MCCC2 3-Methylglutaconic aciduria type I; 250950; AUH 3-Methylglutaconic aciduria
List_of_OMIM_disorder_codes
Austrian pediatric oncologist and metabolism researcher
maint: DOI inactive as of May 2026 (link) "A single mutation in MCCC1 or MCCC2 as a potential cause for positive screening for 3-methylcrotonyl-CoA carboxylase
Raphael_Morscher
MCCC2
MCCC2
MCCC2
MCCC2
Boy/Male
Celtic American English
From the bright valley.
Boy/Male
Biblical
The gift of the Lord.
Surname or Lastname
English
English : occupational name for a hunter, Old English hunta (a primary derivative of huntian ‘to hunt’). The term was used not only of the hunting on horseback of game such as stags and wild boars, which in the Middle Ages was a pursuit restricted to the ranks of the nobility, but also to much humbler forms of pursuit such as bird catching and poaching for food. The word seems also to have been used as an Old English personal name and to have survived into the Middle Ages as an occasional personal name. Compare Huntington and Huntley.Irish : in some cases (in Ulster) of English origin, but more commonly used as a quasi-translation of various Irish surnames such as Ó Fiaich (see Fee).Possibly an Americanized spelling of German Hundt.
Girl/Female
German, Hebrew, Indian, Parsi, Tamil, Turkish
Wind; Flower; Women; Alive; Living
Girl/Female
Arabic, Muslim
Fresh Air
Girl/Female
Afghan, Arabic, Muslim, Pashtun
Brightness of Fire
Girl/Female
Indian
Wind
Boy/Male
British, English, Irish, Latin
Star of the Sea; Small; Form of Paul
Girl/Female
American, Australian, British, Czechoslovakian, English, Finnish, Greek, Latin
Flourishing; Derived from Victoria; Triumphant; Twin; Beyond Price; Invaluable
Surname or Lastname
English
English : variant spelling Musselwhite.
MCCC2
MCCC2
MCCC2
MCCC2
MCCC2