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Biology subjects

Goebel, E. J.

Publications and source records attributed to Goebel, E. J..

5 recordsLinked to original sources

The crystal structure of the activin B:Fst288 complex and computational insights into the broad antagonistic activity and specificity of follistatin

Members of the TGF-{beta} family regulate diverse biological processes, and their activity is tightly controlled by extracellular antagonists such as follistatin 288 (Fst288). Fst288 potently inhibits activins A and B, GDF8, and GDF11. Among these, ActB is the least structurally and functionally characterized, which limits a full understanding of Fst288s broad activity and its specificity for this TGF-{beta} subgroup. To address this, we solved the crystal structure of the ActB:Fst288 complex. As in other complexes, two Fst288 molecules surround ActB, occluding both receptor-binding sites. However, ActB engages Fst288 differently: its fingers bind most strongly the ND domain, while its acidic fingertips uniquely contact FSD3 disrupting interactions between the two Fst288 molecules and reducing the cooperativity seen in other complexes. Computational analysis revealed that, although ActB exhibits higher interaction enthalpy, entropic penalty lowers its overall affinity compared to ActA, consistent with experimentally measured Kd values. We therefore propose that broad ligand inhibition by Fst288 arises from variations in binding interactions and the utilization of cooperativity when direct contacts are insufficient for high affinity. In cellular contexts, antagonistic effectiveness is further impacted by interactions between complexes and the extracellular matrix, resulting in comparable in vitro IC50 values. Finally, sequence comparisons with non-binding TGF-{beta}s indicate that Fst288 specificity originates from ionic contacts with acidic ligand fingertips which likely initiate recognition, while interactions at the type I and II interfaces stabilize the complex. This study provides deeper insight into Fst288s regulation of activin signaling and paves the way for designing inhibitors with desired selectivity.

molecular biology↗

CryoEM structure of ALK2:BMP6 reveals distinct mechanisms that allow ALK2 to interact with both BMP and Activin ligands

Ligands in the TGF{beta} family (activins, BMPs and TGF{beta}s), signal by bringing together two type I and two type II receptors. ALK2 is the only type I receptor among the seven TGF{beta} type I receptors that interacts with both activin and BMP ligands. With BMPs, ALK2 acts as a signaling receptor to activate SMAD1/5/8 signaling. Alternatively, with activins, such as Activin A (ActA), ALK2 forms nonsignaling complexes that negatively regulate ALK2 and ActA signaling. To gain insight into how ALK2 interacts with two distinct classes of ligands, we resolved the cryoelectron microscopy structure of ALK2 in complex with the type II receptor, ActRIIB, and the ligand, BMP6, in parallel with the corresponding structure with ALK3 for direct comparison. These structures demonstrate that ALK2 and ALK3 utilize different mechanisms to interact with BMP6 at the wrist interface, with ALK2 relying on BMP6 glycosylation and ALK3 relying on a salt bridge. Modeling of ALK2:ActA reveals that binding relies on ActAs fingertip region, mirroring the interaction of ActA with its other receptor, ALK4. Our results demonstrate that ALK2 is a hybrid receptor that incorporates features of BMP type I receptors such as ALK3 at the wrist interface, and an activin type I receptor such as ALK4 at the fingertip. SignificanceALK2 interacts with two different classes of TGF{beta} ligands: the BMPs and the activins. With BMP ligands, ALK2 signals to play roles in bone modeling, while with activins, ALK2 negatively regulates signaling by forming nonsignaling complexes. We demonstrate that ALK2 binds to favored ligand, BMP6, by utilizing the ligand wrist-helix stabilized by a glycosylation, rather than a charged interaction observed with similar receptor, ALK3. We also show that ALK2 interaction with activin ligand, ActA, is reliant on a single interaction at the other side of the receptor - the fingertip interface. Thus, this study elucidates first structure of ALK2 in complex with a ligand and provides the molecular insight into how ALK2 is able to bind to BMP and activin ligands.

biochemistry↗

Lysosomal degradation of ACVR1-Activin complexes negatively regulates signaling of Activins and Bone Morphogenetic Proteins

BMP/TGF{beta} family ligands act mainly as factors that differentially initiate Smad1/5/8 or Smad2/3 signaling via heterotetrameric complexes comprised of two type I and two type II receptors (IIR). ActA (ActA) stands out as it activates Smad2/3 signaling through type I receptor ACVR1B, whereas it generates non-signaling complexes (NSCs) with ACVR1. In fibrodysplasia ossificans progressiva (FOP), a genetic disorder caused by missense mutations in ACVR1 (ACVR1.FOP), ACVR1.FOP*ActA*IIR complexes activate Smad1/5 signaling, mimicking those formed with BMPs. As the NSCs that ActA forms with ACVR1 are stoichiometrically identical with signaling complexes formed with ACVR1.FOP, we explored how NSCs differ from their signaling counterparts. We show that ACVR1*ActA*IIR complexes rapidly traffic to lysosomes, where their constituent components are degraded, reducing the cells responsiveness to BMPs along with ActAs availability. This property is specific to ActA as Activin B, AB, and AC do not form lysosomal trafficking complexes with ACVR1, but rather remain on the surface as NSCs. Lysosomal trafficking and degradation of ACVR1*ActA*IIR complexes is a novel regulatory mechanism of BMP/TGF{beta} signaling whose physiological roles remain largely unexplored.

cell biology↗

The orphan ligand, Activin C, signals through activin receptor-like kinase 7

Activin ligands are formed from two disulfide-linked inhibin {beta} subunit chains. They exist as homodimeric proteins, as in the case of activin A (ActA; Inh{beta}A/Inh{beta}A) or activin C (ActC; Inh{beta}C/Inh{beta}C), or as heterodimers, as with activin AC (ActAC; Inh{beta}A:Inh{beta}C). While the biological functions of ActA and activin B (ActB) have been well-characterized, little is known about the biological function of ActC or ActAC. One thought is that the Inh{beta}C chain functions to interfere with ActA production by forming less active ActAC heterodimers. Here, we assessed and characterized the signaling capacity of ligands containing the Inh{beta}C chain. ActC and ActAC activated SMAD2/3-dependent signaling via the type I receptor, activin receptor-like kinase 7 (ALK7). Relative to ActA and ActB, ActC exhibited lower affinity for the cognate activin type II receptors and was resistant to neutralization by the extracellular antagonist, follistatin. In mature adipocytes, which exhibit high ALK7 expression, ActC elicited a SMAD2/3 response similar to ActB, which can also signal via ALK7. Collectively, these results establish that ActC and ActAC are active ligands that exhibit a distinct signaling receptor and antagonist profile compared to other activins.

biochemistry↗

The finger 2 tip loop of Activin A is required for the formation of its non-signaling complex with ACVR1 and type II Bone Morphogenetic Protein receptors

Activin A functions in BMP signaling in two ways: it either engages ACVR1B to activate Smad2/3 signaling or binds ACVR1 to form a non-signaling complex (NSC). Although the former property has been studied extensively, the roles of the NSC remain unexplored. The genetic disorder fibrodysplasia ossificans progressiva (FOP) provides a unique window into ACVR1/Activin A signaling because in that disease Activin can either signal through FOP-mutant ACVR1 or form NSCs with wild type ACVR1. To explore the role of the NSC, we generated agonist-only Activin A muteins that activate ACVR1B but cannot form the NSC with ACVR1. Using one of these muteins we demonstrate that failure to form the NSC in FOP results in more severe disease pathology. These results provide the first evidence for a biological role for the NSC in vivo and pave the way for further exploration of the NSCs physiological role in corresponding knock-in mice. Impact StatementThe non-signaling complex formed by Activin A and ACVR1 is operant in vivo and is required to temper the degree of heterotopic ossification in the genetic disorder fibrodysplasia ossificans progressiva.

cell biology↗