Jeffrey A. Golden

Targeted loss of Arx results in a developmental epilepsy mouse model and recapitulates the human phenotype in heterozygous females (2009)

Marsh, Eric, Fulp, Carl, Gomez, Ernest, Nasrallah, Ilya, Minarcik, Jeremy, Sudi, Jyotsna, ...

Mutations in the X-linked aristaless-related homeobox gene (ARX) have been linked to structural brain anomalies as well as multiple neurocognitive deficits. The generation of Arx-deficient mice...

Mutations of CASK cause an X-linked brain malformation phenotype with microcephaly and hypoplasia of the brainstem and cerebellum (2008)

Najm, Juliane, Horn, Denise, Wimplinger, Isabella, Golden, Jeffrey A, Chizhikov, Victor V, Sudi, Jyotsna, ...

CASK is a multi-domain scaffolding protein that interacts with the transcription factor TBR1 and regulates expression of genes involved in cortical development such as RELN. Here we describe a...

Pax6-/- Mice Have a Cell Nonautonomous Defect in Nonradial Interneuron Migration (2008)

Gopal, Pallavi P., Golden, Jeffrey A.

The mammalian neocortex comprises two major neuronal subtypes; interneurons derived from the ganglionic eminence (GE) and projection neurons from the cortical ventricular zone (VZ). These separate...

Identification of Arx transcriptional targets in the developing basal forebrain (2008)

Fulp, Carl T., Cho, Ginam, Marsh, Eric D., Nasrallah, Ilya M., Labosky, Patricia A., Golden, Jeffrey A.

Mutations in the aristaless-related homeobox (ARX) gene are associated with multiple neurologic disorders in humans. Studies in mice indicate Arx plays a role in neuronal progenitor proliferation and...

Ectopic bone morphogenetic proteins 5 and 4 in the chicken forebrain lead to cyclopia and holoprosencephaly

Golden, Jeffrey A., Bracilovic, Ana, McFadden, Kathryn A., Beesley, Jacqueline S., Rubenstein, John L.R., Grinspan, Judith B.

Proper dorsal–ventral patterning in the developing central nervous system requires signals from both the dorsal and ventral portions of the neural tube. Data from multiple studies have demonstrated...

Intrapulmonary Concentrations of Pyrazinamide

Conte, John E., Golden, Jeffrey A., Duncan, Sheila, McKenna, Elaine, Zurlinden, Elisabeth

The objective of this study was to compare the steady-state plasma and intrapulmonary concentrations of orally administered pyrazinamide in normal volunteers and subjects with AIDS. Pyrazinamide was...

Single-Dose Intrapulmonary Pharmacokinetics of Rifapentine in Normal Subjects

Conte, John E., Golden, Jeffrey A., McQuitty, Mari, Kipps, Juliana, Lin, Emil T., Zurlinden, Elisabeth

The intrapulmonary pharmacokinetics of rifapentine were studied in 30 volunteers who received a single, oral dose of rifapentine (600 mg). Subgroups of five subjects each underwent bronchoscopy and...

Effects of AIDS and Gender on Steady-State Plasma and Intrapulmonary Ethionamide Concentrations

Conte, John E., Golden, Jeffrey A., McQuitty, Mari, Kipps, Juliana, Lin, Emil T., Zurlinden, Elisabeth

Ethionamide, 250 mg every 12 h for a total of nine doses, was administered to 40 adult volunteers (10 men with AIDS, 10 healthy men, 10 women with AIDS, and 10 healthy women). Blood was obtained for...

Effects of AIDS and Gender on Steady-State Plasma and Intrapulmonary Ethambutol Concentrations

Conte, John E., Golden, Jeffrey A., Kipps, Juliana, Lin, Emil T., Zurlinden, Elisabeth

Our objective was to study the steady-state plasma and intrapulmonary orally administered ethambutol concentrations in healthy volunteers and subjects with AIDS. Ethambutol (15 mg/kg of body weight)...

Intrapulmonary Pharmacokinetics of Linezolid

Conte, John E., Golden, Jeffrey A., Kipps, Juliana, Zurlinden, Elisabeth

In this study, our objective was to determine the steady-state intrapulmonary concentrations and pharmacokinetic parameters of orally administered linezolid in healthy volunteers. Linezolid (600 mg...

Effects of Gender, AIDS, and Acetylator Status on Intrapulmonary Concentrations of Isoniazid

Conte Jr., John E., Golden, Jeffrey A., McQuitty, Mari, Kipps, Juliana, Duncan, Sheila, McKenna, Elaine, ...

The objective of the present study was to evaluate the effects of gender, AIDS, and acetylator status on the steady-state concentrations of orally administered isoniazid in plasma and lungs....

Steady-State Plasma and Intrapulmonary Pharmacokinetics and Pharmacodynamics of Cethromycin

Conte, John E., Golden, Jeffrey A., Kipps, Juliana, Zurlinden, Elisabeth

The objective of this study was to determine the steady-state plasma and intrapulmonary pharmacokinetic parameters of orally administered cethromycin in healthy volunteers. The study design included...

Intrapulmonary Pharmacokinetics and Pharmacodynamics of Itraconazole and 14-Hydroxyitraconazole at Steady State

Conte, John E., Golden, Jeffrey A., Kipps, Juliana, McIver, Marina, Zurlinden, Elisabeth

We determined the steady-state intrapulmonary pharmacokinetic and pharmacodynamic parameters of orally administered itraconazole (ITRA), 200 mg every 12 h (twice a day [b.i.d.]), on an empty stomach,...

Pneumocystis carinii Pneumonia Treated With α-Difluoromethylornithine—A Prospective Study Among Patients With the Acquired Immunodeficiency Syndrome

Golden, Jeffrey A., Sjoerdsma, Albert, Santi, Daniel V.

Pneumocystis carinii pneumonia is a protozoal infection that, in the setting of acquired immunodeficiency syndrome (AIDS), is often lethal and unresponsive to conventional therapy with...

A Novel Variant of Inpp5f Is Imprinted in Brain, and Its Expression Is Correlated with Differential Methylation of an Internal CpG Island

Choi, Jonathan D., Underkoffler, Lara A., Wood, Andrew J., Collins, Joelle N., Williams, Patrick T., Golden, Jeffrey A., ...

Using a tissue-specific microarray screen in combination with chromosome anomalies in the mouse, we identified a novel imprinted gene, Inpp5f_v2 on mouse chromosome 7. Characterization of this gene...

Ectopic bone morphogenetic proteins 5 and 4 in the chicken forebrain lead to cyclopia and holoprosencephaly

Golden, Jeffrey A., Bracilovic, Ana, McFadden, Kathryn A., Beesley, Jacqueline S., Rubenstein, John L.R., Grinspan, Judith B.

Proper dorsal–ventral patterning in the developing central nervous system requires signals from both the dorsal and ventral portions of the neural tube. Data from multiple studies have demonstrated...

Intrapulmonary Concentrations of Pyrazinamide

Conte, John E., Golden, Jeffrey A., Duncan, Sheila, McKenna, Elaine, Zurlinden, Elisabeth

The objective of this study was to compare the steady-state plasma and intrapulmonary concentrations of orally administered pyrazinamide in normal volunteers and subjects with AIDS. Pyrazinamide was...

Single-Dose Intrapulmonary Pharmacokinetics of Rifapentine in Normal Subjects

Conte, John E., Golden, Jeffrey A., McQuitty, Mari, Kipps, Juliana, Lin, Emil T., Zurlinden, Elisabeth

The intrapulmonary pharmacokinetics of rifapentine were studied in 30 volunteers who received a single, oral dose of rifapentine (600 mg). Subgroups of five subjects each underwent bronchoscopy and...

Effects of AIDS and Gender on Steady-State Plasma and Intrapulmonary Ethionamide Concentrations

Conte, John E., Golden, Jeffrey A., McQuitty, Mari, Kipps, Juliana, Lin, Emil T., Zurlinden, Elisabeth

Ethionamide, 250 mg every 12 h for a total of nine doses, was administered to 40 adult volunteers (10 men with AIDS, 10 healthy men, 10 women with AIDS, and 10 healthy women). Blood was obtained for...

Effects of AIDS and Gender on Steady-State Plasma and Intrapulmonary Ethambutol Concentrations

Conte, John E., Golden, Jeffrey A., Kipps, Juliana, Lin, Emil T., Zurlinden, Elisabeth

Our objective was to study the steady-state plasma and intrapulmonary orally administered ethambutol concentrations in healthy volunteers and subjects with AIDS. Ethambutol (15 mg/kg of body weight)...

Intrapulmonary Pharmacokinetics of Linezolid

Conte, John E., Golden, Jeffrey A., Kipps, Juliana, Zurlinden, Elisabeth

In this study, our objective was to determine the steady-state intrapulmonary concentrations and pharmacokinetic parameters of orally administered linezolid in healthy volunteers. Linezolid (600 mg...

Effects of Gender, AIDS, and Acetylator Status on Intrapulmonary Concentrations of Isoniazid

Conte Jr., John E., Golden, Jeffrey A., McQuitty, Mari, Kipps, Juliana, Duncan, Sheila, McKenna, Elaine, ...

The objective of the present study was to evaluate the effects of gender, AIDS, and acetylator status on the steady-state concentrations of orally administered isoniazid in plasma and lungs....

Steady-State Plasma and Intrapulmonary Pharmacokinetics and Pharmacodynamics of Cethromycin

Conte, John E., Golden, Jeffrey A., Kipps, Juliana, Zurlinden, Elisabeth

The objective of this study was to determine the steady-state plasma and intrapulmonary pharmacokinetic parameters of orally administered cethromycin in healthy volunteers. The study design included...

Intrapulmonary Pharmacokinetics and Pharmacodynamics of Itraconazole and 14-Hydroxyitraconazole at Steady State

Conte, John E., Golden, Jeffrey A., Kipps, Juliana, McIver, Marina, Zurlinden, Elisabeth

We determined the steady-state intrapulmonary pharmacokinetic and pharmacodynamic parameters of orally administered itraconazole (ITRA), 200 mg every 12 h (twice a day [b.i.d.]), on an empty stomach,...

Pneumocystis carinii Pneumonia Treated With α-Difluoromethylornithine—A Prospective Study Among Patients With the Acquired Immunodeficiency Syndrome

Golden, Jeffrey A., Sjoerdsma, Albert, Santi, Daniel V.

Pneumocystis carinii pneumonia is a protozoal infection that, in the setting of acquired immunodeficiency syndrome (AIDS), is often lethal and unresponsive to conventional therapy with...

A Novel Variant of Inpp5f Is Imprinted in Brain, and Its Expression Is Correlated with Differential Methylation of an Internal CpG Island

Choi, Jonathan D., Underkoffler, Lara A., Wood, Andrew J., Collins, Joelle N., Williams, Patrick T., Golden, Jeffrey A., ...

Using a tissue-specific microarray screen in combination with chromosome anomalies in the mouse, we identified a novel imprinted gene, Inpp5f_v2 on mouse chromosome 7. Characterization of this gene...

PIP5KIγ is required for cardiovascular and neuronal development

Wang, Yanfeng, Lian, Lurong, Golden, Jeffrey A., Morrisey, Edward E., Abrams, Charles S.

All eukaryotic cells contain the phospholipid phosphatidylinositol 4, 5-bisphosphate (PIP2) that serves multiple roles in signal transduction cascades. Type I phosphatidylinositol-4-phosphate...

A polyalanine tract expansion in Arx forms intranuclear inclusions and results in increased cell death

Nasrallah, Ilya M., Minarcik, Jeremy C., Golden, Jeffrey A.

A growing number of human disorders have been associated with expansions of a tract of a single amino acid. Recently, polyalanine (polyA) tract expansions in the Aristaless-related homeobox (ARX)...

Proteomic identification of FHL1 as the protein mutated in human reducing body myopathy

Schessl, Joachim, Zou, Yaqun, McGrath, Meagan J., Cowling, Belinda S., Maiti, Baijayanta, Chin, Steven S., ...

Reducing body myopathy (RBM) is a rare disorder causing progressive muscular weakness characterized by aggresome-like inclusions in the myofibrils. Identification of genes responsible for RBM by...

Lis1 Is Necessary for Normal Non-Radial Migration of Inhibitory Interneurons

McManus, Matthew F., Nasrallah, Ilya M., Pancoast, MacLean M., Wynshaw-Boris, Anthony, Golden, Jeffrey A.

Type I lissencephaly is a central nervous system (CNS) malformation characterized by mental retardation and epilepsy. These clinical features suggest a deficit in inhibitory neurons may, in part,...

Sizn1 Is a Novel Protein That Functions as a Transcriptional Coactivator of Bone Morphogenic Protein Signaling▿ §

Cho, Ginam, Lim, Youngshin, Zand, Dina, Golden, Jeffrey A.

Bone morphogenic proteins (BMPs) play pleotrophic roles in nervous system development, and their signaling is highly regulated at virtually every step in the pathway. We have cloned a novel gene,...

Identification of Arx transcriptional targets in the developing basal forebrain

Fulp, Carl T., Cho, Ginam, Marsh, Eric D., Nasrallah, Ilya M., Labosky, Patricia A., Golden, Jeffrey A.

Mutations in the aristaless-related homeobox (ARX) gene are associated with multiple neurologic disorders in humans. Studies in mice indicate Arx plays a role in neuronal progenitor proliferation and...

Intrapulmonary Pharmacokinetics and Pharmacodynamics of Posaconazole at Steady State in Healthy Subjects▿

Conte, John E., Golden, Jeffrey A., Krishna, Gopal, McIver, Marina, Little, Emily, Zurlinden, Elisabeth

We evaluated the pharmacokinetics (PK) and pharmacodynamics (PD) of posaconazole (POS) in a prospective, open-label study. Twenty-five healthy adults received 14 doses of POS oral suspension (400 mg...