Human Mutation - 2004

229 articles | Last updated: 2025-12-03 14:12:57
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Identification of 26 new constitutional<i>RB1</i>gene mutations in Spanish, Colombian, and Cuban retinoblastoma patients Spanish, Colombian, and Cuban
NovelTMC1 structural and splice variants associated with congenital nonsyndromic deafness in a Sudanese pedigree
Wolfram syndrome in French population: Characterization of novel mutations and polymorphisms in theWFS1 gene
Identification and functional characterization of five novel mutant alleles in 58 Italian patients with Gaucher disease type 1
Kallmann syndrome: 14 novel mutations in<i>KAL1</i>and<i>FGFR1</i>(<i>KAL2</i>)
The mutational spectrum of<i>ENPP1</i>as arising after the analysis of 23 unrelated patients with generalized arterial calcification of infancy (GACI)
The molecular basis of classic Ehlers-Danlos syndrome: A comprehensive study of biochemical and molecular findings in 48 unrelated patients
HAEdb: A novel interactive, locus-specific mutation database for the C1 inhibitor gene
The most common mutation inFKRP causing limb girdle muscular dystrophy type 2I (LGMD2I) may have occurred only once and is present in Hutterites and other populations
DNMT3B mutations and DNA methylation defect define two types of ICF syndrome
Breakpoint analysis of the pericentric inversion distinguishing human chromosome 4 from the homologous chromosome in the chimpanzee (<i>Pan troglodytes</i>)
Phenotology of disease-linked proteins
Variations in human HM74 (GPR109B) and HM74A (GPR109A) niacin receptors
<i>KCNJ11</i>activating mutations in Italian patients with permanent neonatal diabetes Italian
Reassessment of theTP53 mutation database in human disease by data mining with a library ofTP53 missense mutations
A T3 allele in theCFTR gene exacerbates exon 9 skipping in vas deferens and epididymal cell lines and is associated with Congenital Bilateral Absence of Vas Deferens (CBAVD)
Molecular and muscle pathology in a series of caveolinopathy patients
Nonsense-mediated and nonstop decay of ribosomal protein S19 mRNA in Diamond-Blackfan anemia
A novel mutation in UDP-<i>N</i>-acetylglucosamine-1-phosphotransferase gamma subunit (GNPTAG) in two siblings with mucolipidosis type III alters a used glycosylation site
Enchondromatosis (Ollier disease, Maffucci syndrome) is not caused by the PTHR1 mutation p.R150C
LDL-receptor mutations in Europe
Mutations of the Nogo-66 receptor (RTN4R) gene in schizophrenia
Transfer of a mitochondrial DNA fragment toMCOLN1 causes an inherited case of mucolipidosis IV
Haplotypes of the alpha-1 antitrypsin gene in healthy controls and Z deficiency patients
A common nonsense mutation in the repetitive Kringle IV-2 domain of human apolipoprotein(a) results in a truncated protein and low plasma Lp(a)
Identification and characterization of novel mutations of the major Fanconi anemia gene FANCA in the Japanese population
Mutations in the<i>MMAA</i>gene in patients with the<i>cblA</i>disorder of vitamin B<sub>12</sub>metabolism
LargeBRCA1 gene deletions are found in 3% of German high-risk breast cancer families
High-Density SNP genotyping defines 17 distinct haplotypes of the TNF block in the Caucasian population: Implications for haplotype tagging
Functional characterization of SIX3 homeodomain mutations in holoprosencephaly: Interaction with the nuclear receptor NR4A3/NOR1
Disruption of exonic splicing enhancer elements is the principal cause of exon skipping associated with seven nonsense or missense alleles of NF1
Six novel alleles identified in Italian hereditary fructose intolerance patients enlarge the mutation spectrum of the aldolase B gene
A novel mutation in KCNA1 causes episodic ataxia without myokymia
Erratum: Four novel mutations in patients from the Middle East with the infantile form of GM1-gangliosidosis
Genotype-phenotype correlation in von Hippel-Lindau families with renal lesions
ABCC6 mutations in Italian families affected by pseudoxanthoma elasticum (PXE)
Homeologous recombination between AluSx-sequences as a cause of hemophilia
Mutation analysis of<i>NR0B2</i>among 1545 Danish men identifies a novel c.278G&gt;A (p.G93D) variant with reduced functional activity
CRB1 mutation spectrum in inherited retinal dystrophies
Germline mutations of thePOU6F2 gene in Wilms tumors with loss of heterozygosity on chromosome 7p14
Mechanisms underlying responsiveness to tetrahydrobiopterin in mild phenylketonuria mutations
Lack of correlation between the type of COL1A1 or COL1A2 mutation and hearing loss in osteogenesis imperfecta patients
Mutation analysis ofNR2E3 andNRL genes in Enhanced S Cone Syndrome
A point mutation in the lariat branch point of intron 6 ofNPC1 as the cause of abnormal pre-mRNA splicing in Niemann-Pick type C disease
Spectrum ofPTCH mutations in Italian nevoid basal cell-carcinoma syndrome patients: Identification of thirteen novel alleles
Fine mapping and identification of a candidate geneSSH1 in disseminated superficial actinic porokeratosis
A paradigm for single nucleotide polymorphism analysis: The case of the acetylcholinesterase gene
Familial adenomatous polyposis: Aberrant splicing due to missense or silent mutations in the<i>APC</i>gene
Molecular analysis of SMA patients without homozygous SMN1 deletions using a new strategy for identification of SMN1 subtle mutations
Polymorphism analysis within the HLA-A locus by universal oligonucleotide array
Novel and recurrent mutations clustered in the von Willebrand factor A domain of MATN3 in multiple epiphyseal dysplasia
The CDKN2A database: Integrating allelic variants with evolution, structure, function, and disease association
GJB2: The spectrum of deafness-causing allele variants and their phenotype
Molecular and functional analysis ofSLC25A20 mutations causing carnitine-acylcarnitine translocase deficiency
The role of tau (MAPT) in frontotemporal dementia and related tauopathies
Sequence variations of the ?-globin genes: Scanning of high CG content genes with DHPLC and DG-DGGE
Novel homozygous p.E64D mutation in DJ1 in early onset Parkinson disease (PARK7)
Stability related bias in residues replacing glycines within the collagen triple helix (Gly-Xaa-Yaa) in inherited connective tissue disorders
Erratum: Mutational analysis ofPMP22, MPZ, GJB1, EGR2 andNEFL in Korean Charcot-Marie-Tooth neuropathy patients
BRCA1 andBRCA2 germline mutations in Korean patients with sporadic breast cancer
A novel aspartylglucosaminuria mutation affects translocation of aspartylglucosaminidase
Germline mutations inMLH1,MSH2 andMSH6 in Korean hereditary non-polyposis colorectal cancer families
Ten novel<i>MSH2</i>and<i>MLH1</i>germline mutations in families with HNPCC
Four novel mutations in patients from the Middle East with the infantile form of GM1-gangliosidosis
The cystathionine ?-synthase (CBS) mutation c.1224-2A&gt;C in Central Europe: Vitamin B6 nonresponsiveness and a common ancestral haplotype Central Europe
Identification of novel and rare mutations in California Hispanic and African American cystic fibrosis patients
Germline MUTYH (MYH) mutations in Portuguese individuals with multiple colorectal adenomas
RNA analysis reveals splicing mutations and loss of expression defects inMLH1 andBRCA1
IL1RAPL1 is associated with mental retardation in patients with complex glycerol kinase deficiency who have deletions extending telomeric ofDAX1
Splice-site genetic polymorphism of the human kallikrein 12 (KLK12) gene correlates with no substantial expression of KLK12 protein having serine protease activity
Erratum: Novel MC1R variants in ligurian melanoma patients and controls
Carbonic anhydrase II deficiency syndrome (osteopetrosis with renal tubular acidosis and brain calcification): Novel mutations in CA2 identified by direct sequencing expand the opportunity for genotyp
Evidence for mutational cis-acting factors affecting mutagenesis in the ornithine transcarbamylase gene
Impaired calmodulin binding of myosin-7A causes autosomal dominant hearing loss (DFNA11)
Erratum:BMPR2 mutations found in Japanese patients with familial and sporadic primary pulmonary hypertension
Possible gene dosage effect of glutathione-S-transferases on atopic asthma: Using real-time PCR for quantification of GSTM1 and GSTT1 gene copy numbers
Patterns of CAG repeat interruptions inSCA1 andSCA2 genes in relation to repeat instability
TCIRG1-dependent recessive osteopetrosis: Mutation analysis, functional identification of the splicing defects, andin vitro rescue by U1 snRNA
The humanFOXL2 mutation database
Identification and molecular characterization of α-L-iduronidase mutations present in mucopolysaccharidosis type I patients undergoing enzyme replacement therapy
Genotype-phenotype correlation in von Hippel-Lindau families with renal lesions
Reconstruction of patrilineages and matrilineages of Samaritans and other Israeli populations from Y-Chromosome and mitochondrial DNA sequence Variation
Identification of<i>APC</i>gene mutations in colorectal cancer using universal microarray-based combinatorial sequencing-by-hybridization
Infevers: An evolving mutation database for auto-inflammatory syndromes
Acid sphingomyelinase: Identification of nine novel mutations among Italian Niemann Pick type B patients and characterization of<i>in vivo</i>functional in-frame start codon
USH2A Mutation analysis in 70 Dutch families with Usher syndrome type II
Sjögren-Larsson syndrome: Seven novel mutations in the fatty aldehyde dehydrogenase gene ALDH3A2
Characterization of three myotonia-associated mutations of the CLCN1 chloride channel gene via heterologous expression
Mutational analysis ofPMP22, MPZ, GJB1, EGR2 andNEFL in Korean Charcot-Marie-Tooth neuropathy patients
Molecular characterization of familial hypercholesterolemia in Spain: Identification of 39 novel and 77 recurrent mutations in LDLR
Mucopolysaccharidosis IVA (Morquio A): Identification of novel common mutations in the N-acetylgalactosamine-6-sulfate sulfatase (GALNS) gene in Italian patients
A gel-free SNP genotyping method: bioluminometric assay coupled with modified primer extension reactions (BAMPER) directly from double-stranded PCR products
Bayesian approach to discovering pathogenic SNPs in conserved protein domains
The role of common single-nucleotide polymorphisms on exon 9 and exon 12 skipping in nonmutatedCFTR Alleles
Real-time quantitative PCR as a routine method for screening large rearrangements in Rett syndrome: Report of one case of MECP2 deletion and one case of MECP2 duplication
OrthoDisease: A database of human disease orthologs
Lack of correlation between the type of<i>COL1A1</i>or<i>COL1A2</i>mutation and hearing loss in osteogenesis imperfecta patients
Identification of the molecular defect in patients with peroxisomal mosaicism using a novel method involving culturing of cells at 40°C: Implications for other inborn errors of metabolism
CASRdb: calcium-sensing receptor locus-specific database for mutations causing familial (benign) hypocalciuric hypercalcemia, neonatal severe hyperparathyroidism, and autosomal dominant hypocalcemia
Comprehensive molecular screening of the<i>FBN1</i>gene favors locus homogeneity of classical Marfan syndrome
Improved testing for CMT1A and HNPP using multiplex ligation-dependent probe amplification (MLPA) with rapid DNA preparations: Comparison with the interphase FISH Method
Novel MC1R variants in Ligurian melanoma patients and controls
X-linked spondyloepiphyseal dysplasia tarda: Novel and recurrent mutations in 13 European families European families
TBX5 mutations in Non-Holt-Oram Syndrome (HOS) malformed hearts
Maternal MTHFR variant forms increase the risk in offspring of isolated nonsyndromic cleft lip with or without cleft palate
Screening of 25 Italian patients with Niemann-Pick a reveals fourteen new mutations, one common and thirteen private, inSMPD1
A CD36 nonsense mutation associated with insulin resistance and familial type 2 diabetes
Harmonized microarray/mutation scanning analysis of TP53 mutations in undissected colorectal tumors
Two-color multiplex ligation-dependent probe amplification: Detecting genomic rearrangements in hereditary multiple exostoses
Chromosomal anomalies on 6p25 in iris hypoplasia and Axenfeld-Rieger syndrome patients defined on a purpose-built genomic microarray
Molecular screening ofSHH,ZIC2,SIX3, andTGIF genes in patients with features of holoprosencephaly spectrum: Mutation review and genotype-phenotype correlations
RT-PCR permits simultaneous genotyping of thiopurine S-methyltransferase allelic variants by multiplex induced heteroduplex analysis
Molecular diagnosis in LGMD2A: Mutation analysis or protein testing?
The CHEK2 c.1100delC mutation plays an irrelevant role in breast cancer predisposition in Italy
Functional analysis of polymorphisms in the promoter regions of genes on 22q11
Haplotypes of the angiotensin II receptor genesAGTR1 andAGTR2 in women with normotensive pregnancy and women with preeclampsia
Effect of the codon 72 polymorphism (c.215G&gt;C, p.Arg72Pro) in combination with somatic sequence variants in the<i>TP53</i>gene on survival in patients with advanced ovarian carcinoma
Characterization of a mutagenic B1 retrotransposon insertion in the jittery mouse
DFold: PCR design that minimizes secondary structure and optimizes downstream genotyping applications
The androgen receptor gene mutations database (ARDB): 2004 update
Erratum: Genetic and biochemical analyses of Israeli osteogenesis imperfecta patients
Novel and recurrent mutations in theNF1 gene in Italian patients with neurofibromatosis type 1
Seven novel mutations of theADAR gene in Chinese families and sporadic patients with dyschromatosis symmetrica hereditaria (DSH)
Familial melanoma, pancreatic cancer and germline CDKN2A mutations
Detection of 53 novel DNA variations within the tyrosinase gene and accumulation of mutations in 17 patients with albinism
Identification and functional analysis of two novel mutations in the CBS gene in Polish patients with homocystinuria
<i>GJB2</i>mutations in patients with non-syndromic hearing loss from Northeastern Hungary
BMPR2 mutations found in Japanese patients with familial and sporadic primary pulmonary hypertension
NovelGNE mutations in Italian families with autosomal recessive hereditary inclusion-body myopathy
An improved electronic microarray-based diagnostic assay for identification ofMEFV mutations
General implications for CpG hot spot mutations: Methylation patterns of the human iduronate-2-sulfatase gene locus
Functional analysis of 13<i>GBA</i>mutant alleles identified in Gaucher disease patients: Pathogenic changes and “modifier” polymorphisms
Structural and functional analysis of mutations at the human hypoxanthine phosphoribosyl transferase (HPRT1) locus
Branchio-oto-renal syndrome: The mutation spectrum in<i>EYA1</i>and its phenotypic consequences
Denaturing high-performance liquid chromatography (DHPLC) as a reliable high-throughput prescreening method for aberrant promoter methylation in cancer
Molecular and functional analysis ofSUMF1 mutations in multiple sulfatase deficiency
Identification of a new complementation group of the peroxisome biogenesis disorders andPEX14 as the mutated gene
PromoLign: A database for upstream region analysis and SNPs
Transport, enzymatic activity, and stability of mutant sulfamidase (SGSH) identified in patients with mucopolysaccharidosis type III A
Identification and characterization of ANKK1: A novel kinase gene closely linked to DRD2 on chromosome band 11q23.1
The androgen receptor gene mutations database (ARDB): 2004 update
Identification of a functional mutation in pp32r1 (ANP32C)
DNA analysis by MALDI-TOF mass spectrometry
The challenge of documenting mutation across the genome: The human genome variation society approach
The Swiss-Prot variant page and the ModSNP database: A resource for sequence and structure information on human protein variants
Approaches for analyzing human mutations and nucleotide sequence variation: A report from the Seventh International Mutation Detection meeting, 2003
MLPA and MAPH: New techniques for detection of gene deletions
Single-molecule analysis for molecular haplotyping
PCR-Based detection of minority point mutations
Proofreading genotyping assays and electrochemical detection of SNPs
Real-time PCR for single-cell genotyping in sickle cell and thalassemia syndromes as a rapid, accurate, reliable, and widely applicable protocol for preimplantation genetic diagnosis
An activated 5? cryptic splice site in the human ALG3 gene generates a premature termination codon insensitive to nonsense-mediated mRNA decay in a new case of congenital disorder of glycosylation typ
Identification of a novel mutation in the coding region of the grey-lethal gene<i>OSTM1</i>in human malignant infantile osteopetrosis
PAP: Detection of ultra rare mutations depends on P* oligonucleotides: ?Sleeping Beauties? awakened by the kiss of pyrophosphorolysis
Novel mutations in the<i>TRIM37</i>gene in Mulibrey Nanism
Mutation analysis of the adenomatous polyposis coli (<i>APC</i>) gene in Danish patients with familial adenomatous polyposis (FAP)
A novel splice-site mutation in the common gamma chain (?c) geneIL2RG results in X-linked severe combined immunodeficiency with an atypical NK+ phenotype
Molecular analysis of the ABCA4 gene in Turkish patients with Stargardt disease and retinitis pigmentosa
First genotype characterization of Argentinean FAP patients: Identification of 14 novel<i>APC</i>mutations
<b>Characterization of seven novel mutations in seven patients with GAMT deficiency</b>
Detection of<i>Parkin</i>(<i>PARK2</i>) and<i>DJ1</i>(<i>PARK7</i>) mutations in early-onset Parkinson disease:<i>Parkin</i>mutation frequency depends on ethnic origin of patients ethnic origin
Allelic heterogeneity of SMARD1 at the IGHMBP2 locus
Loss-of-function mutations in cathepsin C in two families with Papillon-Lefèvre syndrome are associated with deficiency of serine proteinases in PMNs
Human vitamin K-dependent<i>GAS6</i>: Gene structure, allelic variation, and association with stroke
OCRLMutation analysis in Italian patients with Lowe syndrome Italian patients
Intronic mutations in the<i>L1CAM</i>gene may cause X-linked hydrocephalus by aberrant splicing
<i>PKHD1</i>mutations in families requesting prenatal diagnosis for autosomal recessive polycystic kidney disease (ARPKD)
Genetic characterization of myeloperoxidase deficiency in Italy
<i>PKHD1</i>mutations in autosomal recessive polycystic kidney disease (ARPKD)
Genomic rearrangements in the<i>CFTR</i>gene: Extensive allelic heterogeneity and diverse mutational mechanisms
Leber congenital amaurosis: Comprehensive survey of the genetic heterogeneity, refinement of the clinical definition, and genotype-phenotype correlations as a strategy for molecular diagnosis
Genetically heterogeneous selective intestinal malabsorption of vitamin B<sub>12</sub>: Founder effects, consanguinity, and high clinical awareness explain aggregations in Scandinavia and the Middle E
Identification of a novel candidate gene,<i>CASC2</i>, in a region of common allelic loss at chromosome 10q26 in human endometrial cancer
Large deletions of the MECP2 gene detected by gene dosage analysis in patients with Rett syndrome
Biochemical characterization of two GALK1 mutations in patients with galactokinase deficiency
Molecular analysis of the<i>APC</i>and<i>MYH</i>genes in Czech families affected by FAP or multiple adenomas: 13 novel mutations
Genetic and biochemical analyses of Israeli osteogenesis imperfecta patients
CYLD mutation causes multiple familial trichoepithelioma in three Chinese families Chinese
HAMP gene mutation c.208T&gt;C (p.C70R) identified in an Italian patient with severe hereditary hemochromatosis Italian
Rapid identification of female carriers of DMD/BMD by quantitative real-time PCR
Analysis of the allele-specific expression of the mismatch repair gene<i>MLH1</i>using a simple DHPLC-Based Method
Rapid detection of subtelomeric deletion/duplication by novel real-time quantitative PCR using SYBR-green dye
Genetic variants ofABCA1 modify Alzheimer disease risk and quantitative traits related to ?-amyloid metabolism
Importance of standard nomenclature forSMN1 small intragenic (?subtle?) mutations
Genetic variants in a haplotype block spanningIDE are significantly associated with plasma A?42 levels and risk for Alzheimer disease
Molecular screening of<i>ALK1/ACVRL1</i>and<i>ENG</i>genes in hereditary hemorrhagic telangiectasia in France
Variant late infantile neuronal ceroid lipofuscinosis in a subset of Turkish patients is allelic to Northern epilepsy
Bridging structural biology and genetics by computational methods: An investigation into how the R774C mutation in the AR gene can result in complete androgen insensitivity syndrome
Identification of four novel COL10A1 missense mutations in schmid metaphyseal chondrodysplasia: Further evidence that collagen X NC1 mutations impair trimer assembly
NovelTSC2 mutations and decreased expression of tuberin in cultured tumor cells with an insertion mutation
BRCA1 and BRCA2 mutations in women with familial or early-onset breast/ovarian cancer in the Czech Republic
Wilson disease: Novel mutations in theATP7B gene and clinical correlation in Brazilian patients
Identification of the bruton tyrosine kinase (BTK) gene mutations in 20 Australian families with X-linked agammaglobulinemia (XLA)
The west side story:<i>MEFV</i>haplotype in Spanish FMF patients and controls, and evidence of high LD and a recombination “hot-spot” at the<i>MEFV</i>locus
Mutational analysis of mucopolysaccharidosis type VI patients undergoing a trial of enzyme replacement therapy
The role of cathepsin C in Papillon-Lefèvre syndrome, prepubertal periodontitis, and aggressive periodontitis
Molecular basis of Refsum disease: Sequence variations in Phytanoyl-CoA Hydroxylase (PHYH) and the PTS2 receptor (PEX7)
Analysis of CBP (CREBBP) gene deletions in Rubinstein-Taybi syndrome patients using real-time quantitative PCR
A nicotine<i>C</i>-oxidase gene (<i>CYP2A6</i>) polymorphism important for promoter activity
APECED-causing mutations in AIRE reveal the functional domains of the protein
Large deletions of the MECP2 gene detected by gene dosage analysis in patients with Rett syndrome
Noonan syndrome-associated SHP2/PTPN11 mutants cause EGF-dependent prolonged GAB1 binding and sustained ERK2/MAPK1 activation
Gross rearrangement breakpoint database (GRaBD?)
Eight novel<i>MSH6</i>germline mutations in patients with familial and nonfamilial colorectal cancer selected by loss of protein expression in tumor tissue
Molecular characterization of familial hypercholesterolemia in German and Greek patients
BTK: 22 novel and 25 recurrent mutations in European patients with X-linked agammaglobulinemia European patients
A homozygousGJA1 gene mutation causes a Hallermann-Streiff/ODDD spectrum phenotype
Novel androgen receptor gene mutations in Australian patients with complete androgen insensitivity syndrome
Novel <i>TP53</i> gene mutations in tumors of Russian patients with breast cancer detected using a new solid phase chemical cleavage of mismatch method and identified by sequen
Screening 500 unselected neurofibromatosis 1 patients for deletions of the <i>NF1</i> gene
The pedigree tool: Web‐based visualization of a family tree
Loss of function of the cytoplasmic isoform of the protein laforin (EPM2A) causes Lafora progressive myoclonus epilepsy
Mutations of <i>ARX</i> are associated with striking pleiotropy and consistent genotype–phenotype correlation
Mutations in the MATP gene in five German patients affected by oculocutaneous albinism type 4
Context of deletions and insertions in human coding sequences
Two novel severe mutations in the pancreatic secretory trypsin inhibitor gene ( <i>SPINK1</i> ) cause familial and/or hereditary pancreatitis
Novel germline mutations in the <i>BRCA1</i> and <i>BRCA2</i> genes in Indian breast and breast‐ovarian cancer families
The molecular basis of cystathionine ß‐synthase (CBS) deficiency in UK and US patients with homocystinuria
Mutation spectrum in Taiwanese patients with phenylalanine hydroxylase deficiency and a founder effect for the R241C mutation
Low frequency of deafness‐associated <i>GJB2</i> variants in Kenya and Sudan and novel <i>GJB2</i> variants
Y‐chromosomal microsatellite mutation rates: Differences in mutation rate between and within loci
Comprehensive screening for constitutional <i>RB1</i> mutations by DHPLC and QMPSF
Mitochondrial DNA‐like sequences in the nucleus (NUMTs): Insights into our African origins and the mechanism of foreign DNA integration
Characterization of the somatic mutational spectrum of the neurofibromatosis type 1 ( <i>NF1</i> ) gene in neurofibromatosis patients with benign and malignant tumors
Three‐tiered noninvasive diagnosis in 96% of patients with Duchenne muscular dystrophy (DMD)
Genetic characterization and structural analysis of VHL Spanish families to define genotype–phenotype correlations
Five novel mutations in the lysosomal sialidase gene (<i>NEU1</i>) in type II sialidosis patients and assessment of their impact on enzyme activity and intracellular targeting using adenovirus-mediate
Knobloch syndrome: Novel mutations in<i>COL18A1</i>, evidence for genetic heterogeneity, and a functionally impaired polymorphism in endostatin
Interaction between trypsinogen isoforms in genetically determined pancreatitis: Mutation E79K in cationic trypsin (PRSS1) causes increased transactivation of anionic trypsinogen (PRSS2)