FBP1 - fructose-bisphosphatase 1 Gene

Homo sapiens

Also known as FBP

Gene ID: 2203 | Gene type: protein coding

About FBP1

Cytogenetic location: 9q22.32 Genomic coordinates (GRCh38): 9:94,603,133-94,640,263 (from NCBI)

This gene has 5 transcripts (splice variants), 143 orthologues, 1 paralogue and is associated with 3 phenotypes. Biased expression in liver (RPKM 175.9), kidney (RPKM 118.4) and 9 other tissues.

Summary

Fructose-1,6-bisphosphatase 1, a gluconeogenesis regulatory Enzyme, catalyzes the hydrolysis of fructose 1,6-bisphosphate to fructose 6-phosphate and inorganic phosphate. Fructose-1,6-diphosphatase deficiency is associated with hypoglycemia and metabolic acidosis. [provided by RefSeq, Jul 2008]

FBP1 Products(2)

mRNA	Protein	Name
NM_000507.4	NP_000498.2	fructose-1,6-bisphosphatase 1
NM_001127628.2	NP_001121100.1	fructose-1,6-bisphosphatase 1

Gene Ontology

Molecular Function
Biological Process
Cellular Component

Molecular Function GO Annotation	Evidence	Reference	Source
enables AMP binding	IDA IDA: Inferred from direct assay	8387495	GOA
enables RNA polymerase II-specific DNA-binding transcription factor binding	IMP IMP: Inferred from mutant phenotype	25043030	GOA
enables fructose 1,6-bisphosphate 1-phosphatase activity	IDA IDA: Inferred from direct assay	8387495	GOA
enables fructose 1,6-bisphosphate 1-phosphatase activity	IMP IMP: Inferred from mutant phenotype	7558035	GOA
enables identical protein binding	IPI IPI: Inferred from physical interaction	16169070	GOA
enables metal ion binding	IMP IMP: Inferred from mutant phenotype	8387495	GOA
enables protein binding	IPI IPI: Inferred from physical interaction	8387495	GOA

Biological Process GO Annotation	Evidence	Reference	Source
involved in cellular response to magnesium ion	IDA IDA: Inferred from direct assay	10222032	GOA
involved in cellular response to xenobiotic stimulus	IDA IDA: Inferred from direct assay	16442285	GOA
involved in dephosphorylation	IDA IDA: Inferred from direct assay	8387495	GOA
involved in dephosphorylation	IMP IMP: Inferred from mutant phenotype	7558035	GOA
involved in fructose 6-phosphate metabolic process	IDA IDA: Inferred from direct assay	8387495	GOA
involved in gluconeogenesis	IMP IMP: Inferred from mutant phenotype	19259699	GOA
involved in negative regulation of Ras protein signal transduction	IDA IDA: Inferred from direct assay	19881551	GOA
involved in negative regulation of cell growth	IDA IDA: Inferred from direct assay	19881551	GOA
involved in negative regulation of glycolytic process	IDA IDA: Inferred from direct assay	19881551	GOA
involved in negative regulation of glycolytic process	IMP IMP: Inferred from mutant phenotype	25043030	GOA
involved in negative regulation of transcription by RNA polymerase II	IMP IMP: Inferred from mutant phenotype	25043030	GOA
involved in regulation of gluconeogenesis	IMP IMP: Inferred from mutant phenotype	7558035	GOA

Cellular Component GO Annotation	Evidence	Reference	Source
located in cytoplasm	IDA IDA: Inferred from direct assay	20096900	GOA
located in nucleus	IMP IMP: Inferred from mutant phenotype	25043030	GOA

EXP: Inferred from Experiment IDA: Inferred from direct assay IPI: Inferred from physical interaction IMP: Inferred from mutant phenotype IGI: Inferred from genetic interaction IEP: Inferred from expression pattern

FBP1 Protein Structure

FBPase

0
100
200
300
338 a.a.

Protein Preferred Names

Protein Names

fructose-1,6-bisphosphatase 1

D-fructose-1,6-bisphosphate 1-phosphohydrolase 1

Recombinant FBP1 Proteins

Cat. No.	Product Name	Accession	Purity
HY-P70275	FBP1 Protein, Human (HEK293, His)	P09467 (A2-Q338)	≥95%

Related Diseases

Diseases

Alias

Fructose-1,6-Bisphosphatase Deficiency

Fructose-1,6-Diphosphatase Deficiency	Baker-Winegrad Disease
FBP1D	Fbpase Deficiency
Fructose 1,6 Diphosphatase Deficiency	Fructose 1 Phosphate Aldolase Deficiency
Fbp1 Deficiency	Hereditary Fructose Intolerance Syndrome

Hypoglycemia

Hypoglycaemia	Low Blood Sugar
Hypoglycaemia Nos	Spontaneous Hypoglycaemia
Nondiabetic Hypoglycaemia	Hypoglycaemic Disorder Nos
Hypoglycaemic Syndrome

Metabolic Acidosis

Lactic Acidosis

Acidosis, Lactic

Acidosis Lactic

Acute Pyelonephritis

Apn - [Acute Pyelonephritis]

Glycogen Storage Disease Ia

Von Gierke Disease	Glycogen Storage Disease Type I
Glycogen Storage Disease I	Hepatorenal Form Of Glycogen Storage Disease
Hepatorenal Glycogenosis	Glucose-6-Phosphatase Deficiency
Glycogen Storage Disease, Type I	Glycogen Storage Disease Due To Glucose-6-Phosphatase Deficiency Type Ia
GSD1A	Gsd1
Von Gierke'S Disease	Glycogen Storage Disease Type 1a
Glycogen Storage Disease 1a	Glucose-6-Phosphate Transport Defect
Gsd Ia	Deficiency Of Glucose-6-Phosphatase
Glycogenosis Type I	Glucose-6-Phosphatase Deficiency Glycogen Storage Disease
Glycogenosis Type 1	Glucose-6-Phosphate Deficiency
Gsd I	Gsd Type I
G6p Deficiency Type 1a	Gsd Due To G6p Deficiency Type 1a
Gsd Due To G6p Deficiency Type Ia	Gsd Type 1a
Gsdia	Glycogen Storage Disease Due To G6p Deficiency Type Ia
Glycogenosis Due To Glucose-6-Phosphatase Deficiency Type 1a	Glycogenosis Due To Glucose-6-Phosphatase Deficiency Type Ia
Glycogenosis Type Ia	Gsd-Ia
Storage Disease, Glycogen, Type 1a	Glycogen Storage Disease Type Ia

Hepatocellular Carcinoma

Liver Cancer	Primary Liver Cancer
HCC	Hepatoma
Malignant Neoplasm Of Liver	Liver Neoplasms
Cancer, Hepatocellular	Liver Cell Carcinoma
Lcc	Hepatoblastoma, Somatic
Hepatic Cancer	Primary Malignant Neoplasm Of Liver
Rare Tumor Of Liver And Intrahepatic Biliary Tract	Hepatocellular Carcinoma, Somatic
Hepatocellular Carcinoma, Childhood Type, Somatic	Hepatocellular Cancer, Somatic
Ca Liver - Primary	Hepatic Neoplasm
Malignant Hepato-Biliary Neoplasm	Malignant Neoplasm Of Liver, Not Specified As Primary Or Secondary
Malignant Neoplasm Of Liver, Primary	Malignant Tumor Of Liver
Neoplasm Of Liver	Non-Resectable Primary Hepatic Malignant Neoplasm
Resectable Malignant Neoplasm Of Liver	Resectable Malignant Neoplasm Of The Liver
Primary Liver Carcinoma	Primary Malignant Liver Neoplasm
Primary Cancer Of Liver	Primary Tumor Of The Liver
Rare Tumor Of Liver And Ibt	Hepatocellular Cancer
Neoplasm Of The Liver	Carcinoma, Hepatocellular
Hepatomas	Liver Neoplasm
Liver Carcinoma	Liver And Intrahepatic Biliary Tract Carcinoma
Malignant Hepatobiliary Neoplasm	Adult Primary Hepatocellular Carcinoma
Hepatoblastoma	Carcinoma Of Liver
Malignant Liver Tumour	Malignant Hepatic Tumour

Diseases	Alias
Waldenstroem'S Macroglobulinemia	Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma Waldenstroem'S Macroglobulinemia Waldenstroem'S Macroglobulinemia Macroglobulinemia Of Waldenstrom Lymphoplasmacytic Lymphoma With Igm Gammopathy Lymphoplasmacytic Lymphoma

Related Agents

Pre-clinical Phase

Bioactive Molecules (1)

Cat. No.	Product Name	Effect	Purity	Rare Diseases
HY-RS04779	FBP1 Human Pre-designed siRNA Set A	Pre-designed Sets	/	Unkown

Orthologs Information

Species	Symbol	Source	ID
Mus musculus	FBP1	MGD	MGI:95492
Rattus norvegicus	FBP1	RGD	RGD:2595
Bos taurus	FBP1	VGNC	VGNC:28887
Felis catus	FBP1	VGNC	VGNC:62172
Canis familiaris	FBP1	VGNC	VGNC:40757
Macaca mulatta	FBP1	VGNC	VGNC:72625
Others	FBP1	NCBI

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