Type II Progressive Familial Heart Block

Also Known As:

Progressive Familial Heart Block, Type II; PFHB2; PFHBII

Networked: 4 relevant articles (0 outcomes, 0 trials/studies)

Disease Context: Research Results

Cardiovascular Diseases: 91362
- Heart Diseases: 26014
  - Cardiac Arrhythmias: 20790
    - Heart Block: 1547
      - Type II Progressive Familial Heart Block: 4
  - Cardiac Conduction System Disease: 93
    - Heart Block: 1547
      - Type II Progressive Familial Heart Block: 4
Signs and Symptoms Pathological Conditions
- Pathologic Processes: 10023
  - Cardiac Arrhythmias: 20790
    - Heart Block: 1547
      - Type II Progressive Familial Heart Block: 4

Experts

1.	Mattoo, Seema: 2 articles (01/2018 - 09/2011)
2.	Hedrick, Victoria: 1 article (01/2018)
3.	Kumar, Sanjeev: 1 article (01/2018)
4.	Adler, Ben: 1 article (01/2016)
5.	Boyce, John D: 1 article (01/2016)
6.	Harper, Marina: 1 article (01/2016)
7.	Harrison, Paul: 1 article (01/2016)
8.	Kleifeld, Oded: 1 article (01/2016)
9.	Mégroz, Marianne: 1 article (01/2016)
10.	Powell, David: 1 article (01/2016)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Type II Progressive Familial Heart Block:

1.	Proteins (Proteins, Gene)FDA Link 01/01/2018 - "Here, a catalytically inactive C3733S mutant of PfhB2 YopT that provides enhanced protein stability was used with the aim of gaining structural insight into the diversity within the YopT protein family. " 01/01/2018 - "YopT domain of the PfhB2 toxin from Pasteurella multocida: protein expression, characterization, crystallization and crystallographic analysis." 09/16/2011 - "We also included the Fic domains of the secreted protein, PfhB2, from the opportunistic pathogen Pasteurella multocida, in our analysis. " 01/01/2016 - "Both the transcript and protein levels for genes/proteins involved in capsule biosynthesis were reduced in the hfq mutant, as were the levels of the filamentous hemagglutinin protein PfhB2 and its secretion partner LspB2. "
2.	Cysteine Proteases (Cysteine Protease)IBA 01/01/2018 - "multocida secretes a multidomain toxin called PfhB2, which contains a YopT-like cysteine protease domain at its C-terminus. "
3.	rho GTP-Binding Proteins (rho GTP-Binding Protein)IBA 09/16/2011 - "We demonstrate that the PfhB2 Fic domains also possess adenylyltransferase activity that targets the switch 1 tyrosine of Rho GTPases. " 09/16/2011 - "Comparative kinetic and phylogenetic analyses of IbpA-Fic2 with the Fic domains of PfhB2, VopS, and HYPE reveal important aspects of their specificities for Rho GTPases and nucleotide usage and offer mechanistic insights for determining nucleotide and substrate specificities for these enzymes. "
4.	Troponin T (Troponin T1)IBA 10/01/2005 - "This region encompassed the troponin T gene (TNNT2), however, genetic fine mapping and haplotype analysis excluded TNNT2 as cause of PFHBII and placed the disease-causative gene within a 3.9 cM (2.85 Mb) interval, flanked by D1S70 and D1S505. "
5.	Tyrosine (L-Tyrosine)FDA Link 09/16/2011 - "We demonstrate that the PfhB2 Fic domains also possess adenylyltransferase activity that targets the switch 1 tyrosine of Rho GTPases. "
6.	Peptide Hydrolases (Proteases)FDA Link 01/01/2018 - "The YopT domain of PfhB2 contains a well conserved Cys-His-Asp catalytic triad that defines YopT family members, and shares high sequence similarity with the prototype YopT from Yersinia sp. To date, only one crystal structure of a YopT family member has been reported; however, additional structural information is needed to help characterize the varied substrate specificity and enzymatic action of this large protease family. "
7.	NucleotidesIBA 09/16/2011 - "Comparative kinetic and phylogenetic analyses of IbpA-Fic2 with the Fic domains of PfhB2, VopS, and HYPE reveal important aspects of their specificities for Rho GTPases and nucleotide usage and offer mechanistic insights for determining nucleotide and substrate specificities for these enzymes. "
8.	Hemagglutinins (Hemagglutinin)IBA 01/01/2016 - "Both the transcript and protein levels for genes/proteins involved in capsule biosynthesis were reduced in the hfq mutant, as were the levels of the filamentous hemagglutinin protein PfhB2 and its secretion partner LspB2. "
9.	EnzymesIBA 09/16/2011 - "Comparative kinetic and phylogenetic analyses of IbpA-Fic2 with the Fic domains of PfhB2, VopS, and HYPE reveal important aspects of their specificities for Rho GTPases and nucleotide usage and offer mechanistic insights for determining nucleotide and substrate specificities for these enzymes. "
10.	Capsules (Microcapsules)IBA 01/01/2016 - "Both the transcript and protein levels for genes/proteins involved in capsule biosynthesis were reduced in the hfq mutant, as were the levels of the filamentous hemagglutinin protein PfhB2 and its secretion partner LspB2. "