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Identification
HMDB Protein ID HMDBP00980
Secondary Accession Numbers
  • 6268
  • HMDBP05972
Name Fructose-bisphosphate aldolase B
Synonyms
  1. Liver-type aldolase
Gene Name ALDOB
Protein Type Enzyme
Biological Properties
General Function Involved in fructose-bisphosphate aldolase activity
Specific Function Not Available
Pathways
  • Fructose and Mannose Degradation
  • Fructose and mannose metabolism
  • Fructose intolerance, hereditary
  • Fructosuria
  • glycolysis
  • Glycolysis / Gluconeogenesis
  • Pentose phosphate pathway
  • Warburg Effect
Reactions
Fructose 1,6-bisphosphate → Dihydroxyacetone phosphate + D-Glyceraldehyde 3-phosphate details
beta-D-Fructose 1,6-bisphosphate → Dihydroxyacetone phosphate + D-Glyceraldehyde 3-phosphate details
Sedoheptulose 1,7-bisphosphate → Dihydroxyacetone phosphate + D-Erythrose 4-phosphate details
Fructose 1-phosphate → Dihydroxyacetone phosphate + Glyceraldehyde details
GO Classification
Biological Process
response to zinc ion
fructose 1,6-bisphosphate metabolic process
cellular response to extracellular stimulus
fructose catabolic process
NADH oxidation
positive regulation of ATPase activity
response to amino acid stimulus
response to fructose stimulus
response to interleukin-6
vacuolar proton-transporting V-type ATPase complex assembly
glycolysis
liver development
response to drug
response to starvation
cellular response to insulin stimulus
gluconeogenesis
response to glucocorticoid stimulus
response to cAMP
response to copper ion
Cellular Component
cytosol
centriolar satellite
rough endoplasmic reticulum membrane
plasma membrane
perinuclear region of cytoplasm
nucleus
lysosome
smooth endoplasmic reticulum membrane
Function
catalytic activity
lyase activity
carbon-carbon lyase activity
aldehyde-lyase activity
fructose-bisphosphate aldolase activity
Molecular Function
fructose binding
fructose-bisphosphate aldolase activity
ATPase binding
cytoskeletal protein binding
phosphatidylcholine binding
Process
metabolic process
small molecule metabolic process
alcohol metabolic process
monosaccharide metabolic process
hexose metabolic process
glucose metabolic process
glucose catabolic process
glycolysis
Cellular Location Not Available
Gene Properties
Chromosome Location 9
Locus 9q21.3-q22.2
SNPs ALDOB
Gene Sequence
>1095 bp
ATGGCCCACCGATTTCCAGCCCTCACCCAGGAGCAGAAGAAGGAGCTCTCAGAAATTGCC
CAGAGCATTGTTGCCAATGGAAAGGGGATCCTGGCTGCAGATGAATCTGTAGGTACCATG
GGGAACCGCCTGCAGAGGATCAAGGTGGAAAACACTGAAGAGAACCGCCGGCAGTTCCGA
GAAATCCTCTTCTCTGTGGACAGTTCCATCAACCAGAGCATCGGGGGTGTGATCCTTTTC
CACGAGACCCTCTACCAGAAGGACAGCCAGGGAAAGCTGTTCAGAAACATCCTCAAGGAA
AAGGGGATCGTGGTGGGAATCAAGTTAGACCAAGGAGGTGCTCCTCTTGCAGGAACAAAC
AAAGAAACCACCATTCAAGGGCTTGATGGCCTCTCAGAGCGCTGTGCTCAGTACAAGAAA
GATGGTGTTGACTTTGGGAAGTGGCGTGCTGTGCTGAGGATTGCCGACCAGTGTCCATCC
AGCCTCGCTATCCAGGAAAACGCCAACGCCCTGGCTCGCTACGCCAGCATCTGTCAGCAG
AATGGACTGGTACCTATTGTTGAACCAGAGGTAATTCCTGATGGAGACCATGACCTGGAA
CACTGCCAGTATGTTACTGAGAAGGTCCTGGCTGCTGTCTACAAGGCCCTGAATGACCAT
CATGTTTACCTGGAGGGCACCCTGCTAAAGCCCAACATGGTGACTGCTGGACATGCCTGC
ACCAAGAAGTATACTCCAGAACAAGTAGCTATGGCCACCGTAACAGCTCTCCACCGTACT
GTTCCTGCAGCTGTTCCTGGCATCTGCTTTTTGTCTGGTGGCATGAGTGAAGAGGATGCC
ACTCTCAACCTCAATGCTATCAACCTTTGCCCTCTACCAAAGCCCTGGAAACTAAGTTTC
TCTTATGGACGGGCCCTGCAGGCCAGTGCACTGGCTGCCTGGGGTGGCAAGGCTGCAAAC
AAGGAGGCAACCCAGGAGGCTTTTATGAAGCGGGCCATGGCTAACTGCCAGGCGGCCAAA
GGACAGTATGTTCACACGGGTTCTTCTGGGGCTGCTTCCACCCAGTCGCTCTTCACAGCC
TGCTATACCTACTAG
Protein Properties
Number of Residues 364
Molecular Weight 39472.715
Theoretical pI 7.867
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Fructose-bisphosphate aldolase B
MAHRFPALTQEQKKELSEIAQSIVANGKGILAADESVGTMGNRLQRIKVENTEENRRQFR
EILFSVDSSINQSIGGVILFHETLYQKDSQGKLFRNILKEKGIVVGIKLDQGGAPLAGTN
KETTIQGLDGLSERCAQYKKDGVDFGKWRAVLRIADQCPSSLAIQENANALARYASICQQ
NGLVPIVEPEVIPDGDHDLEHCQYVTEKVLAAVYKALNDHHVYLEGTLLKPNMVTAGHAC
TKKYTPEQVAMATVTALHRTVPAAVPGICFLSGGMSEEDATLNLNAINLCPLPKPWKLSF
SYGRALQASALAAWGGKAANKEATQEAFMKRAMANCQAAKGQYVHTGSSGAASTQSLFTA
CYTY
GenBank ID Protein 55958631
UniProtKB/Swiss-Prot ID P05062
UniProtKB/Swiss-Prot Entry Name ALDOB_HUMAN
PDB IDs
GenBank Gene ID AL353621
GeneCard ID ALDOB
GenAtlas ID ALDOB
HGNC ID HGNC:417
References
General References
  1. Humphray SJ, Oliver K, Hunt AR, Plumb RW, Loveland JE, Howe KL, Andrews TD, Searle S, Hunt SE, Scott CE, Jones MC, Ainscough R, Almeida JP, Ambrose KD, Ashwell RI, Babbage AK, Babbage S, Bagguley CL, Bailey J, Banerjee R, Barker DJ, Barlow KF, Bates K, Beasley H, Beasley O, Bird CP, Bray-Allen S, Brown AJ, Brown JY, Burford D, Burrill W, Burton J, Carder C, Carter NP, Chapman JC, Chen Y, Clarke G, Clark SY, Clee CM, Clegg S, Collier RE, Corby N, Crosier M, Cummings AT, Davies J, Dhami P, Dunn M, Dutta I, Dyer LW, Earthrowl ME, Faulkner L, Fleming CJ, Frankish A, Frankland JA, French L, Fricker DG, Garner P, Garnett J, Ghori J, Gilbert JG, Glison C, Grafham DV, Gribble S, Griffiths C, Griffiths-Jones S, Grocock R, Guy J, Hall RE, Hammond S, Harley JL, Harrison ES, Hart EA, Heath PD, Henderson CD, Hopkins BL, Howard PJ, Howden PJ, Huckle E, Johnson C, Johnson D, Joy AA, Kay M, Keenan S, Kershaw JK, Kimberley AM, King A, Knights A, Laird GK, Langford C, Lawlor S, Leongamornlert DA, Leversha M, Lloyd C, Lloyd DM, Lovell J, Martin S, Mashreghi-Mohammadi M, Matthews L, McLaren S, McLay KE, McMurray A, Milne S, Nickerson T, Nisbett J, Nordsiek G, Pearce AV, Peck AI, Porter KM, Pandian R, Pelan S, Phillimore B, Povey S, Ramsey Y, Rand V, Scharfe M, Sehra HK, Shownkeen R, Sims SK, Skuce CD, Smith M, Steward CA, Swarbreck D, Sycamore N, Tester J, Thorpe A, Tracey A, Tromans A, Thomas DW, Wall M, Wallis JM, West AP, Whitehead SL, Willey DL, Williams SA, Wilming L, Wray PW, Young L, Ashurst JL, Coulson A, Blocker H, Durbin R, Sulston JE, Hubbard T, Jackson MJ, Bentley DR, Beck S, Rogers J, Dunham I: DNA sequence and analysis of human chromosome 9. Nature. 2004 May 27;429(6990):369-74. [PubMed:15164053 ]
  2. Paolella G, Santamaria R, Izzo P, Costanzo P, Salvatore F: Isolation and nucleotide sequence of a full-length cDNA coding for aldolase B from human liver. Nucleic Acids Res. 1984 Oct 11;12(19):7401-10. [PubMed:6548561 ]
  3. Sakakibara M, Mukai T, Yatsuki H, Hori K: Human aldolase isozyme gene: the structure of multispecies aldolase B mRNAs. Nucleic Acids Res. 1985 Jul 25;13(14):5055-69. [PubMed:2410860 ]
  4. Rottmann WH, Tolan DR, Penhoet EE: Complete amino acid sequence for human aldolase B derived from cDNA and genomic clones. Proc Natl Acad Sci U S A. 1984 May;81(9):2738-42. [PubMed:6585824 ]
  5. Mukai T, Yatsuki H, Arai Y, Joh K, Matsuhashi S, Hori K: Human aldolase B gene: characterization of the genomic aldolase B gene and analysis of sequences required for multiple polyadenylations. J Biochem. 1987 Nov;102(5):1043-51. [PubMed:2830249 ]
  6. Tolan DR, Penhoet EE: Characterization of the human aldolase B gene. Mol Biol Med. 1986 Jun;3(3):245-64. [PubMed:3016456 ]
  7. Sakakibara M, Takahashi I, Takasaki Y, Mukai T, Hori K: Construction and expression of human aldolase A and B expression plasmids in Escherichia coli host. Biochim Biophys Acta. 1989 Apr 12;1007(3):334-42. [PubMed:2649152 ]
  8. Besmond C, Dreyfus JC, Gregori C, Frain M, Zakin MM, Sala Trepat J, Kahn A: Nucleotide sequence of a cDNA clone for human aldolase B. Biochem Biophys Res Commun. 1983 Dec 16;117(2):601-9. [PubMed:6689266 ]
  9. Dalby AR, Tolan DR, Littlechild JA: The structure of human liver fructose-1,6-bisphosphate aldolase. Acta Crystallogr D Biol Crystallogr. 2001 Nov;57(Pt 11):1526-33. Epub 2001 Oct 25. [PubMed:11679716 ]
  10. Tolan DR: Molecular basis of hereditary fructose intolerance: mutations and polymorphisms in the human aldolase B gene. Hum Mutat. 1995;6(3):210-8. [PubMed:8535439 ]
  11. Cross NC, Tolan DR, Cox TM: Catalytic deficiency of human aldolase B in hereditary fructose intolerance caused by a common missense mutation. Cell. 1988 Jun 17;53(6):881-5. [PubMed:3383242 ]
  12. Cross NC, de Franchis R, Sebastio G, Dazzo C, Tolan DR, Gregori C, Odievre M, Vidailhet M, Romano V, Mascali G, et al.: Molecular analysis of aldolase B genes in hereditary fructose intolerance. Lancet. 1990 Feb 10;335(8685):306-9. [PubMed:1967768 ]
  13. Brooks CC, Tolan DR: A partially active mutant aldolase B from a patient with hereditary fructose intolerance. FASEB J. 1994 Jan;8(1):107-13. [PubMed:8299883 ]
  14. Ali M, Cox TM: Diverse mutations in the aldolase B gene that underlie the prevalence of hereditary fructose intolerance. Am J Hum Genet. 1995 Apr;56(4):1002-5. [PubMed:7717389 ]
  15. Ali M, Sebastio G, Cox TM: Identification of a novel mutation (Leu 256-->Pro) in the human aldolase B gene associated with hereditary fructose intolerance. Hum Mol Genet. 1994 Jan;3(1):203-4. [PubMed:8162030 ]
  16. Cross NC, Stojanov LM, Cox TM: A new aldolase B variant, N334K, is a common cause of hereditary fructose intolerance in Yugoslavia. Nucleic Acids Res. 1990 Apr 11;18(7):1925. [PubMed:2336380 ]
  17. Lau J, Tolan DR: Screening for hereditary fructose intolerance mutations by reverse dot-blot. Mol Cell Probes. 1999 Feb;13(1):35-40. [PubMed:10024431 ]
  18. Santamaria R, Esposito G, Vitagliano L, Race V, Paglionico I, Zancan L, Zagari A, Salvatore F: Functional and molecular modelling studies of two hereditary fructose intolerance-causing mutations at arginine 303 in human liver aldolase. Biochem J. 2000 Sep 15;350 Pt 3:823-8. [PubMed:10970798 ]
  19. Sanchez-Gutierrez JC, Benlloch T, Leal MA, Samper B, Garcia-Ripoll I, Feliu JE: Molecular analysis of the aldolase B gene in patients with hereditary fructose intolerance from Spain. J Med Genet. 2002 Sep;39(9):e56. [PubMed:12205126 ]
  20. Esposito G, Santamaria R, Vitagliano L, Ieno L, Viola A, Fiori L, Parenti G, Zancan L, Zagari A, Salvatore F: Six novel alleles identified in Italian hereditary fructose intolerance patients enlarge the mutation spectrum of the aldolase B gene. Hum Mutat. 2004 Dec;24(6):534. [PubMed:15532022 ]
  21. Santer R, Rischewski J, von Weihe M, Niederhaus M, Schneppenheim S, Baerlocher K, Kohlschutter A, Muntau A, Posselt HG, Steinmann B, Schneppenheim R: The spectrum of aldolase B (ALDOB) mutations and the prevalence of hereditary fructose intolerance in Central Europe. Hum Mutat. 2005 Jun;25(6):594. [PubMed:15880727 ]