These mice contain a global knock-out of the sulfonylurea receptor (Sur1), which is part of the ATP-dependent potassium channel.  In humans, mutations in Sur1 cause persistent hyperinsulinemic hypoglycemia of infancy (PHHI).


Record History
Added on July 19, 2010 at 10:17 AM by Magnuson, Mark
Modified on April 14, 2017 at 1:14 PM by Skelton, Jennifer
Shared with (contributions)
Public: Shared Collection

Mouse Information

Common Name Sur1neo
Research Applications Not provided
Jackson Laboratories Stock No Not provided
Additional Strain Information Not provided

Genetic Alteration

Mutation #1: Targeted Mutagenesis
Type of AlleleGlobal Null
Targeted GeneName: ATP-binding cassette, sub-family C (CFTR/MRP), member 8
Symbol: Abcc8
NCBI: 20927
Allele Name: targeted mutation 1.1Mgn
Symbol: Sur1neotm1.1Mgn
MGI: MGI:2388392
Description of Targeting Vector

A gene targeting strategy that involved the use of Cre/loxP was used generate mice that globally lack the proximal promoter and exon 1 of the sulfonylurea receptor type 1 (Sur1) gene. A pgk-neomycin resistance gene remains in the locus. Genotype: DNA PCR utilizing 5'-CAA TTC CTC AAC TGA GGC TCT TAA-3' and 5'-AGC CTC TGT TCC ACA TAC ACT TCA-3' primers amplify a 414 bp Sur1[neo] allele. DNA PCR using 5'-CAA TTC CTC AAC TGA GGC TCT TAA-3' and 5'-TCG CAG AGT GAC CTC ACA GCC TGT-3' primers amplify a 412 bp for the wild type allele. Homozygous phenotype: Mice that are homozygous null for Sur1 (Abcc8) are viable, fertile and grossly indistinguishable from their wild type littermates except after 16 wks when they become mildly hypoglycemia. Mice lacking Sur1 (Abcc8) lack functional K-ATP channels in pancreatic beta cells but remain euglycemic despite having beta cells that are constantly depolarized due to the lack of this protein. Heterozygous phenotype: These mice are also viable and do not differ from wild type.

Vector Genbank File
Allele Map Not Provided
PCR Genotyping Protocol Not provided
Sulfonylurea receptor type 1 knock-out mice have intact feeding-stimulated insulin secretion despite marked impairment in their response to glucose. (2002) J Biol Chem 277: 37176-83 (Added 12/10/2013)
PMID: 12149271

Background Strain Information

Strain Type Congenic Strain
Chimera/Founder Genetic Background 129S6/SvEvTac
Current Genetic Background C57BL/6J
Number of Generations Backcrossed 10
Strain Description

After achieving germline transmission mice carrying the Sur1[neo] allele were backcrossed for ten generations.

Publications / Citations

  1. Differential structure of atrial and ventricular KATP: atrial KATP channels require SUR1. Flagg TP, Kurata HT, Masia R, Caputa G, Magnuson MA, Lefer DJ, Coetzee WA, Nichols CG (2008) Circ Res 103(12): 1458-65
    › Primary publication · 18974387 (PubMed) · PMC2768594 (PubMed Central) · Added on 2/23/2011
  2. Exendin-(9-39) corrects fasting hypoglycemia in SUR-1-/- mice by lowering cAMP in pancreatic beta-cells and inhibiting insulin secretion. De León DD, Li C, Delson MI, Matschinsky FM, Stanley CA, Stoffers DA (2008) J Biol Chem 283(38): 25786-93
    › Primary publication · 18635551 (PubMed) · PMC3258866 (PubMed Central) · Added on 12/21/2016
  3. Role of sulfonylurea receptor type 1 subunits of ATP-sensitive potassium channels in myocardial ischemia/reperfusion injury. Elrod JW, Harrell M, Flagg TP, Gundewar S, Magnuson MA, Nichols CG, Coetzee WA, Lefer DJ (2008) Circulation 117(11): 1405-13
    › Primary publication · 18316485 (PubMed) · Added on 2/23/2011
  4. Hyperinsulinism in mice with heterozygous loss of K(ATP) channels. Remedi MS, Rocheleau JV, Tong A, Patton BL, McDaniel ML, Piston DW, Koster JC, Nichols CG (2006) Diabetologia 49(10): 2368-78
    › Primary publication · 16924481 (PubMed) · Added on 12/21/2016
  5. Cholinergic regulation of fuel-induced hormone secretion and respiration of SUR1-/- mouse islets. Doliba NM, Qin W, Vatamaniuk MZ, Buettger CW, Collins HW, Magnuson MA, Kaestner KH, Wilson DF, Carr RD, Matschinsky FM (2006) Am J Physiol Endocrinol Metab 291(3): E525-35
    › Primary publication · 16638820 (PubMed) · Added on 2/23/2011
  6. Impaired glucagon secretory responses in mice lacking the type 1 sulfonylurea receptor. Shiota C, Rocheleau JV, Shiota M, Piston DW, Magnuson MA (2005) Am J Physiol Endocrinol Metab 289(4): E570-7
    › Primary publication · 15941784 (PubMed) · Added on 12/10/2013
  7. Restitution of defective glucose-stimulated insulin release of sulfonylurea type 1 receptor knockout mice by acetylcholine. Doliba NM, Qin W, Vatamaniuk MZ, Li C, Zelent D, Najafi H, Buettger CW, Collins HW, Carr RD, Magnuson MA, Matschinsky FM (2004) Am J Physiol Endocrinol Metab 286(5): E834-43
    › Primary publication · 14736703 (PubMed) · Added on 2/23/2011
  8. Sulfonylurea receptor type 1 knock-out mice have intact feeding-stimulated insulin secretion despite marked impairment in their response to glucose. Shiota C, Larsson O, Shelton KD, Shiota M, Efanov AM, Hoy M, Lindner J, Kooptiwut S, Juntti-Berggren L, Gromada J, Berggren PO, Magnuson MA (2002) J Biol Chem 277(40): 37176-83
    › Primary publication · 12149271 (PubMed) · Added on 12/10/2013
  9. The imidazoline RX871024 stimulates insulin secretion in pancreatic beta-cells from mice deficient in K(ATP) channel function. Efanov AM, Høy M, Bränström R, Zaitsev SV, Magnuson MA, Efendic S, Gromada J, Berggren PO (2001) Biochem Biophys Res Commun 284(4): 918-22
    › Primary publication · 11409880 (PubMed) · Added on 5/27/2010

MeSH Terms

1-Methyl-3-isobutylxanthine Acetylcholine Acetylcholinesterase Amino Acids Animals ATP-Binding Cassette Transporters Blood Glucose Calcium Carbachol Cell Respiration Cells, Cultured Chlorocebus aethiops Cholinergic Fibers Cloning, Molecular COS Cells Cyclic AMP Diabetes Complications Eating Exocytosis Female Fibrosis Gene Expression Gene Expression Regulation Genotype Glucagon Glucagon-Like Peptide-1 Receptor Glucagon-Like Peptide 1 Glucose Glucose Clamp Technique Glucose Transporter Type 2 Glyburide Heart Atria Heart Ventricles Hormones Hyperinsulinism Hypoglycemia Hypoglycemic Agents Imidazoles Indoles Insulin Insulin-Secreting Cells Insulin Secretion Intracellular Fluid In Vitro Techniques Islets of Langerhans KATP Channels Kinetics Kv1.3 Potassium Channel Large-Conductance Calcium-Activated Potassium Channel beta Subunits Loss of Heterozygosity Male Membrane Potentials Mice Mice, Inbred C57BL Mice, Inbred Strains Mice, Knockout Mice, Transgenic Models, Biological Molecular Sequence Data Multidrug Resistance-Associated Proteins Myocardial Infarction Myocardial Reperfusion Injury Myocarditis Myocytes, Cardiac Oocytes Oxygen Consumption Patch-Clamp Techniques Peptide Fragments Perfusion Phosphodiesterase Inhibitors Potassium Channel Blockers Potassium Channels Potassium Channels, Inwardly Rectifying Promoter Regions, Genetic Protein Precursors Receptors, Drug Receptors, Glucagon Recombinant Proteins Reference Values RNA, Messenger Second Messenger Systems Signal Transduction Sulfonylurea Compounds Sulfonylurea Receptors Ventricular Dysfunction, Left Xenopus laevis


Document bc4021079001.jpeg - Added on July 27, 2010 at 9:35 AM by Jill Lindner


Document sur_neo__wt_protocol_l__w.doc - Added on July 27, 2010 at 2:30 PM by Jill Lindner

PCR protocol for genotyping mice