Defects in ankyrin-based cellular pathways in metazoan physiology.

نویسندگان

  • Peter J Mohler
  • Vann Bennett
چکیده

Ankyrins are a ubiquitously expressed family of membrane-adaptor proteins found in most vertebrate tissues. Since the first ankyrin polypeptide was identified over 25 years ago, studies in humans, mice, and lower organisms have implicated critical roles for ankyrins in normal metazoan physiology. This review will provide an overview of the ankyrin family and highlight seminal findings in the field which have linked dysfunction in ankyrin-based pathways with defects in metazoan physiology and human disease.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Spectrin and ankyrin-based pathways: metazoan inventions for integrating cells into tissues.

The spectrin-based membrane skeleton of the humble mammalian erythrocyte has provided biologists with a set of interacting proteins with diverse roles in organization and survival of cells in metazoan organisms. This review deals with the molecular physiology of spectrin, ankyrin, which links spectrin to the anion exchanger, and two spectrin-associated proteins that promote spectrin interaction...

متن کامل

Defining new insight into atypical arrhythmia: a computational model of ankyrin-B syndrome.

Normal cardiac excitability depends on the coordinated activity of specific ion channels and transporters within specialized domains at the plasma membrane and sarcoplasmic reticulum. Ion channel dysfunction due to congenital or acquired defects has been linked to human cardiac arrhythmia. More recently, defects in ion channel-associated proteins have been associated with arrhythmia. Ankyrin-B ...

متن کامل

Defects in Cytoskeletal Signaling Pathways, Arrhythmia, and Sudden Cardiac Death

Ankyrin polypeptides are cellular adapter proteins that tether integral membrane proteins to the cytoskeleton in a host of human organs. Initially identified as integral components of the cytoskeleton in erythrocytes, a recent explosion in ankyrin research has demonstrated that these proteins play prominent roles in cytoskeletal signaling pathways and membrane protein trafficking/regulation in ...

متن کامل

Ankyrins and Spectrins in Cardiovascular Biology and Disease

Ankyrins are adaptor proteins critical for the expression and targeting of cardiac membrane proteins, signaling molecules, and cytoskeletal elements. Findings in humans and animal models have highlighted the in vivo roles for ankyrins in normal physiology and in cardiovascular disease, most notably in cardiac arrhythmia. For example, human ANK2 loss-of-function variants are associated with a co...

متن کامل

Arrhythmia/Electrophysiology Defects in Ankyrin-Based Membrane Protein Targeting Pathways Underlie Atrial Fibrillation

AF. Ankyrin-B is expressed in atrial myocytes, and we demonstrate its requirement for the membrane targeting and function of a subgroup of voltage-gated Ca channels (Cav1.3) responsible for low voltage-activated L-type Ca 2 current. Ankyrin-B is associated directly with Cav1.3, and this interaction is regulated by a short, highly conserved motif specific to Cav1.3. Moreover, loss of ankyrin-B i...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Frontiers in bioscience : a journal and virtual library

دوره 10  شماره 

صفحات  -

تاریخ انتشار 2005