Iris Lindberg
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View article: The neuronal chaperone proSAAS is highly expressed in the retina
The neuronal chaperone proSAAS is highly expressed in the retina Open
The many layers of the neuroretina contain a complex, interconnected network of specialized neurons that both process visual stimuli and conduct processed information to higher brain areas. Neural networks rely on proteostatic control mech…
View article: SEL1L-HRD1 ER-Associated Degradation Facilitates Prohormone Convertase 2 Maturation and Glucagon Production in Islet α Cells
SEL1L-HRD1 ER-Associated Degradation Facilitates Prohormone Convertase 2 Maturation and Glucagon Production in Islet α Cells Open
Proteolytic cleavage of proglucagon by prohormone convertase 2 (PC2) is required for islet α cells to generate glucagon. However, the regulatory mechanisms underlying this process remain largely unclear. Here, we report that SEL1L-HRD1 end…
View article: ProSAAS is preferentially up‐regulated during homeostatic scaling and reduces amyloid plaque burden in the 5xFAD mouse hippocampus
ProSAAS is preferentially up‐regulated during homeostatic scaling and reduces amyloid plaque burden in the 5xFAD mouse hippocampus Open
The accumulation of β‐amyloid in Alzheimer's disease greatly impacts neuronal health and synaptic function. To maintain network stability in the face of altered synaptic activity, neurons engage a feedback mechanism termed homeostatic scal…
View article: ProSAAS is Preferentially Secreted from Neurons During Homeostatic Scaling and Reduces Amyloid Plaque Size in the 5xFAD Mouse Hippocampus
ProSAAS is Preferentially Secreted from Neurons During Homeostatic Scaling and Reduces Amyloid Plaque Size in the 5xFAD Mouse Hippocampus Open
The accumulation of β-amyloid in Alzheimer’s disease greatly impacts neuronal health and synaptic function. To maintain network stability in the face of altered synaptic activity, neurons engage a feedback mechanism termed homeostatic scal…
View article: SERCA2 regulates proinsulin processing and processing enzyme maturation in the pancreatic β cell
SERCA2 regulates proinsulin processing and processing enzyme maturation in the pancreatic β cell Open
Increased circulating levels of incompletely processed insulin (i.e. proinsulin) are observed clinically in both type 1 and type 2 diabetes; however, the mechanisms underlying impaired proinsulin processing remain incompletely understood. …
View article: Sequestration of TDP-43 <sup>216-414</sup> Aggregates by Cytoplasmic Expression of the proSAAS Chaperone
Sequestration of TDP-43 <sup>216-414</sup> Aggregates by Cytoplasmic Expression of the proSAAS Chaperone Open
As neurons age, protein homeostasis becomes less efficient, resulting in misfolding and aggregation. Chaperone proteins perform vital functions in the maintenance of cellular proteostasis, and chaperone-based therapies that promote sequest…
View article: The proSAAS Chaperone Provides Neuroprotection and Attenuates Transsynaptic α-Synuclein Spread in Rodent Models of Parkinson’s Disease
The proSAAS Chaperone Provides Neuroprotection and Attenuates Transsynaptic α-Synuclein Spread in Rodent Models of Parkinson’s Disease Open
Background: Parkinson’s disease involves aberrant aggregation of the synaptic protein alpha-synuclein (aSyn) in the nigrostriatal tract. We have previously shown that proSAAS, a small neuronal chaperone, blocks aSyn-induced dopaminergic cy…
View article: The G209R mutant mouse as a model for human <i>PCSK1</i> polyendocrinopathy
The G209R mutant mouse as a model for human <i>PCSK1</i> polyendocrinopathy Open
PCSK1 encodes an enzyme required for prohormone maturation into bioactive peptides. A striking number of SNPs and rare mutations in PCSK1 are associated with a range of clinical phenotypes. Infants bearing two copies of a catalytically ina…
View article: The proSAAS chaperone provides neuroprotection and attenuates transsynaptic α–synuclein spread in rodent models of Parkinson’s disease
The proSAAS chaperone provides neuroprotection and attenuates transsynaptic α–synuclein spread in rodent models of Parkinson’s disease Open
Parkinson’s disease is a devastating motor disorder involving the aberrant aggregation of the synaptic protein synuclein (aSyn) and degeneration of the nigrostriatal dopaminergic tract. We previously showed that proSAAS, a small secreted c…
View article: Obesity, POMC, and POMC-processing Enzymes: Surprising Results From Animal Models
Obesity, POMC, and POMC-processing Enzymes: Surprising Results From Animal Models Open
Peptides derived from proopiomelanocortin (POMC) are well-established neuropeptides and peptide hormones that perform multiple functions, including regulation of body weight. In humans and some animals, these peptides include α– and β–mela…
View article: Mice lacking PC1/3 expression in POMC-expressing cells do not develop obesity
Mice lacking PC1/3 expression in POMC-expressing cells do not develop obesity Open
Pro-opiomelanocortin (POMC) neurons form an integral part of the central melanocortin system regulating food intake and energy expenditure. Genetic and pharmacological studies have revealed that defects in POMC synthesis, processing, and r…
View article: A protease protection assay for the detection of internalized alpha-synuclein pre-formed fibrils
A protease protection assay for the detection of internalized alpha-synuclein pre-formed fibrils Open
Alpha-synuclein pre-formed fibrils (PFFs) represent a promising model system for the study of cellular processes underlying cell-to-cell transmission of alpha-synuclein proteopathic aggregates. However, the ability to differentiate the fat…
View article: Mouse Models of Human Proprotein Convertase Insufficiency
Mouse Models of Human Proprotein Convertase Insufficiency Open
The kexin-like proprotein convertases perform the initial proteolytic cleavages that ultimately generate a variety of different mature peptide and proteins, ranging from brain neuropeptides to endocrine peptide hormones, to structural prot…
View article: Deorphanization of Novel Peptides and Their Receptors
Deorphanization of Novel Peptides and Their Receptors Open
Peptide hormones and neuropeptides play important roles in endocrine and neural signaling, often using G protein-coupled receptor (GPCR)-mediated signaling pathways. However, the rate of novel peptide discovery has slowed dramatically in r…
View article: A protease protection assay for the detection of internalized alpha-synuclein pre-formed fibrils
A protease protection assay for the detection of internalized alpha-synuclein pre-formed fibrils Open
Alpha-synuclein pre-formed fibrils (PFFs) represent a promising model system for the study of cellular processes underlying cell-to-cell transmission of alpha-synuclein proteopathic aggregates. However, the ability to differentiate the fat…
View article: Secreted Chaperones in Neurodegeneration
Secreted Chaperones in Neurodegeneration Open
Protein homeostasis, or proteostasis, is a combination of cellular processes that govern protein quality control, namely, protein translation, folding, processing, and degradation. Disruptions in these processes can lead to protein misfold…
View article: Increased expression and retention of the secretory chaperone proSAAS following cell stress
Increased expression and retention of the secretory chaperone proSAAS following cell stress Open
The secretory pathway of neurons and endocrine cells contains a variety of mechanisms designed to combat cellular stress. These include not only the unfolded protein response pathways but also diverse chaperone proteins that collectively w…
View article: A comprehensive systematic review of CSF proteins and peptides that define Alzheimer’s disease
A comprehensive systematic review of CSF proteins and peptides that define Alzheimer’s disease Open
Background During the last two decades, over 100 proteomics studies have identified a variety of potential biomarkers in CSF of Alzheimer’s (AD) patients. Although several reviews have proposed specific biomarkers, to date, the statistical…
View article: Sequestration of TDP-43<sup>216-414</sup>aggregates by cytoplasmic expression of the proSAAS chaperone
Sequestration of TDP-43<sup>216-414</sup>aggregates by cytoplasmic expression of the proSAAS chaperone Open
As neurons age, protein homeostasis becomes less efficient, resulting in misfolding and aggregation. Chaperone proteins perform vital functions in the maintenance of cellular proteostasis, and chaperone-based therapies that promote sequest…
View article: SUN-669 The G209R Mutant Mouse as a Model for Human PCSK1 Polyendocrinopathy
SUN-669 The G209R Mutant Mouse as a Model for Human PCSK1 Polyendocrinopathy Open
A striking number of SNPs and rare mutations have been identified in PCSK1, the gene that codes for the enzyme proprotein convertase 1/3 (PC1/3) which proteolytically activates prohormones within the secretory pathway. All infants bearing …
View article: Additional file 2 of A comprehensive systematic review of CSF proteins and peptides that define Alzheimer’s disease
Additional file 2 of A comprehensive systematic review of CSF proteins and peptides that define Alzheimer’s disease Open
Additional file 2: Table S2. Proteins identified with significant changes between AD and control samples. The number of studies that describe these changes are shown.
View article: Additional file 3 of A comprehensive systematic review of CSF proteins and peptides that define Alzheimer’s disease
Additional file 3 of A comprehensive systematic review of CSF proteins and peptides that define Alzheimer’s disease Open
Additional file 3: Table S3. Proteins showing inconsistent changes in AD samples. $ proteins which are abundant in the CSF of healthy individuals [18]. Component CO4A and CO4B are indicated as A and B respectively.
View article: Additional file 4 of A comprehensive systematic review of CSF proteins and peptides that define Alzheimer’s disease
Additional file 4 of A comprehensive systematic review of CSF proteins and peptides that define Alzheimer’s disease Open
Additional file 4: Table S4. Information regarding all peptides extracted from the proteomic studies. M.W. [Da]: Molecular weight obtained from each article. Theo. M.W. [Da]: theoretical molecular weight obtained from Compute pI/Mw tool (h…
View article: Additional file 1 of A comprehensive systematic review of CSF proteins and peptides that define Alzheimer’s disease
Additional file 1 of A comprehensive systematic review of CSF proteins and peptides that define Alzheimer’s disease Open
Additional file 1: Table S1. Information regarding all the proteins extracted from the proteomic studies. The proteins identified in each article and their abbreviations are shown. Δ, proteins not recognized by DAVID bioinformatics resourc…
View article: Additional file 5 of A comprehensive systematic review of CSF proteins and peptides that define Alzheimer’s disease
Additional file 5 of A comprehensive systematic review of CSF proteins and peptides that define Alzheimer’s disease Open
Additional file 5: Table S5. Peptide sequences showing inconsistent changes in CSF studies. Peptide sequences with different pattern of expression across independent studies or inconsistent direction change to that observed by proteomics i…
View article: Physiological signaling in the absence of amidated peptides
Physiological signaling in the absence of amidated peptides Open
Peptidergic signaling is an ancient manner of intertissue communication in multicellular organisms. Even the early eukaryote Trichoplax , with its limited 6-tissue repertoire, uses peptides to communicate between its tissues (1). Humans us…
View article: Reduced stability and pH-dependent activity of a common obesity-linked PCSK1 polymorphism, N221D
Reduced stability and pH-dependent activity of a common obesity-linked PCSK1 polymorphism, N221D Open
Common mutations in the human prohormone convertase 1/3 (PC1/3) gene (PCSK1) are linked to increased risk of obesity. Previous work has shown that the rs6232 SNP (N221D) results in slightly decreased activity, though whether this decrease …