Last update 01 Nov 2024

HBZ

Last update 01 Nov 2024

Basic Info

Synonyms

HBAZ, HBZ, HBZ-T1

+ [5]

Introduction

The zeta chain is an alpha-type chain of mammalian embryonic hemoglobin.

Drugs associated with HBZ

CN117050192

Patent Mining

Target

Chemokine ligands x HBZ

Mechanism-

Active Org.

Xinxiang Medical University

Originator Org.

Xinxiang Medical University

Active Indication

Immune System Diseases

Inactive Indication-

Drug Highest PhaseDiscovery

First Approval Ctry. / Loc.-

First Approval Date20 Jan 1800

100 Clinical Results associated with HBZ

100 Translational Medicine associated with HBZ

0 Patents (Medical) associated with HBZ

438

Literatures (Medical) associated with HBZ

31 Dec 2024·Hematology

Identification of a novel 145 kb deletion (Guigang deletion, – ^Guigang ) in the alpha-globin gene cluster from a Chinese newborn using third-generation sequencing

Article

Author: Li, Yan ; Wei, Wei ; Liang, Liang ; Li, Youqiong ; Li, Teng ; Guo, Jing ; Guo, Weilin

OBJECTIVETo describe a novel α-thalassemiadeletion identified from a newborn by third-generation sequencing (TGS).CASE REPORTThe proband, a newborn subject to neonatal capillary electrophoresis (CE) screening, exhibited suspected α⁰-thalassemia carrier status (Hb Bart's 3.0%). Notably, both parents had negative results on thalassemia screening during pregnancy. Multiplex ligation-dependent probe amplification (MLPA) presented a deletion between probes 364nt and 472 nt that extended from the HBZ gene to the downstream region of the RGS11 gene. Subsequently, TGS determined the approximated break position of this deletion, indicating a length exceeding 145 kb (chr16:127,815-273,190 del 145376 bp). Sanger sequencing validated the upstream and downstream breakpoints of this deletion. Only maternal data were available for pedigree analysis, with the father's sample lacking. MLPA showed no deletion in the mother, suggesting possible paternal inheritance. The deletion was named Guigang deletion (--^Guigang) after the proband's city of origin, Guigang.CONCLUSIONSWe reported a novel α-thalassemiadeletion and provided insights into the hematological phenotype and molecular analysis. These findings have implications for genetic counseling and prenatal diagnosis.

12 Sep 2024·The Journal of Physical Chemistry B

Sickle Cell Hemoglobin “Drugged” with Cyclic Peptides Is Aggregation Incompetent

Article

Author: Galamba, N.

Sickle cell disease (SCD) is a monogenic blood disorder associated with a mutation in the hemoglobin subunit β gene encoding for the β-globin of normal adult hemoglobin (HbA). This mutation transcribes into a Glu-β6 → Val-β6 substitution in the β-globins, inducing the polymerization of this hemoglobin form (HbS) when in the T-state. Despite advances in stem cell and gene therapy, and the recent approval of a new antisickling drug, therapeutic limitations persist. Herein, we demonstrate through molecular dynamics and umbrella sampling, that (unrestrained) blockage of the hydrophobic pocket involved in the lateral contact of the HbS fibers by 5-mer cyclic peptides, recently proposed as SCD aggregation inhibitors (Neto, V.; J. Med. Chem. 2023, 66, 16062-16074), is enough to turn the dimerization of HbS thermodynamically unfavorable. Among these potential drugs, some exhibit an estimated pocket abandonment probability of around 15-20% within the simulations' time frame, and an impressive specificity toward the mutated Val-β6. Additionally, we show that the dimerization can be thermodynamically unfavored by blocking a nearby region while the pocket remains vacant. These results are compared with curcumin, an antisickling molecule and a pan-assay interference compound, with a good binding affinity for different proteins and protein domains. Our results confirm the potential of some of these cyclic peptides as antisickling drug candidates to reduce the concentration of aggregation-competent HbS.

01 Sep 2024·Journal of Medical Virology

Mapping variants in HTLV‐1 genome to analyze their impacts on the HAM/TSP development: A systematic review

Review

Author: Khouri, Ricardo ; Silva, Dhara Isabella Barreto de Souza ; Nascimento, Jéssica Oliveira de Souza ; Santana, Carolina Souza ; Costa, Davi Tanajura ; de Oliveira Andrade, Felipe ; Campos, Raissa Frazão ; Santos, Luciane Amorim ; Barreto, Laura Nascimento ; Barreto, Fernanda Khouri ; Borba, Melina Mosquero Navarro ; Cucco, Marina Silveira ; Lima, Ana Carolina Marinho Monteiro

AbstractThe reasons that lead some individuals living with the Human T Lymphotropic Virus 1 (HTLV‐1) to develop HAM/TSP are still unclear. To better understand the viral genetic factors that may be associated with the development of HAM/TSP, this study aims to evaluate the impact of HTLV‐1 genome mutations on the development of this disease through a systematic review. This review followed the PRISMA guidelines and was registered in the PROSPERO database. The search for articles was performed in PMC, PubMed, Lilacs, SciELO, and Embase databases using the following search descriptors: HTLV‐1, HAM/TSP, mutation, polymorphism, genetic variation, and sequenc*. From the 1,929 articles found in the search, 20 were selected according to the pre‐defined inclusion and exclusion criteria. A total of 619 HAM/TSP cases were compared with 555 AC controls. The mutations possibly related to the disease progression were detected in hbz (R119Q), tax (A7959V), ORF‐I (R88K, P86S, S69G, P45L, L40F, C39R, CR9Y), and gp46 (V247I, N93D, S72G) genetic regions. The data collected and analyzed here indicate that mutations in the HTLV‐1 genome could play an important role in the chronic inflammatory state and may be related to the development of HAM/TSP.

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HBZ

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