Literature DB >> 6787331

Is pyruvate carboxylase involved in the renal tubular reabsorption of bicarbonate?

N R Buist.   

Abstract

The co-existence of a hereditary defect of pyruvate carboxylase activity along with proximal renal tubular acidosis in several patients prompted the following theories: (1) Some of the bicarbonate which is normally reabsorbed from the glomerular filtrate is trapped in the mitochondria by pyruvate carboxylase in the conversion of pyruvate to oxaloacetate. The subsequent conversion of oxaloacetate to phosphoenol pyruvate releases CO2 in the cytosol. (2) The trapping of HCO-3 by pyruvate (or other carboxylases) provides an important route for the recovery of filtered HCO-3. (3) The process of trapping HCO-3 from the glomerular filtrate followed by release of CO2 in the cytosol contributes to the apparently high RQ of kidney, since the CO2 does not originate from a metabolic fuel. (4) Lactate and possibly other fuels are actively taken up by the kidney and are used as energy sources. Diversion of lactate for gluconeogenesis may contribute to the "excess substrate uptake" phenomenon. (5) It is possible that some of the glucose which is synthesized in the cortex is used for glycolysis in the medulla. Conversely, lactate produced in the medulla may be available to the cortex for bicarbonate trapping and thus for gluconeogenesis.

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Year:  1980        PMID: 6787331     DOI: 10.1007/BF02312544

Source DB:  PubMed          Journal:  J Inherit Metab Dis        ISSN: 0141-8955            Impact factor:   4.982


  17 in total

1.  Intracellular localization of carbonic anhydrase in rat liver and kidney tissues.

Authors:  P K DATTA; T H SHEPARD
Journal:  Arch Biochem Biophys       Date:  1959-03       Impact factor: 4.013

2.  Congenital lactic acidosis due to pyruvate carboxylase deficiency: absence of an inhibitor of TPP-ATP phosphoryl transferase.

Authors:  K Tada; G Takada; K Omura; Y Itokawa
Journal:  Eur J Pediatr       Date:  1978-01-17       Impact factor: 3.183

3.  Pyruvate carboxylase deficiency and lactic acidosis in a retarded child without Leigh's disease.

Authors:  B M Atkin; N R Buist; M F Utter; A B Leiter; B Q Banker
Journal:  Pediatr Res       Date:  1979-02       Impact factor: 3.756

4.  Biochemical studies and therapy in subacute necrotizing encephalomyelopathy (Leigh's syndrome).

Authors:  W D Grover; V H Auerbach; M S Patel
Journal:  J Pediatr       Date:  1972-07       Impact factor: 4.406

5.  Pyruvate carboxylase in human liver. Apparent loss of a component of catalytic activity in a form of lactic acidosis with hypoglycemia.

Authors:  E Delvin; C R Scriver; J L Neal
Journal:  Biochem Med       Date:  1974-06

6.  Leigh's encephalomyelopathy: an inborn error of gluconeogenesis.

Authors:  F A Hommes; H A Polman; J D Reerink
Journal:  Arch Dis Child       Date:  1968-08       Impact factor: 3.791

7.  Pathogenesis of Leigh's encephalomyelopathy.

Authors:  T T Tang; T A Good; P R Dyken; S D Johnsen; S R McCreadie; S T Sy; H A Lardy; F B Rudolph
Journal:  J Pediatr       Date:  1972-07       Impact factor: 4.406

8.  Liver and kidney metabolism during prolonged starvation.

Authors:  O E Owen; P Felig; A P Morgan; J Wahren; G F Cahill
Journal:  J Clin Invest       Date:  1969-03       Impact factor: 14.808

9.  Hyperalaninemia hyperpyruvicemia and lactic acidosis due to pyruvate carboxylase deficiency of the liver; treatment with thiamine and lipoic acid.

Authors:  H Maesaka; K Komiya; K Misugi; K Tada
Journal:  Eur J Pediatr       Date:  1976-05-04       Impact factor: 3.183

10.  Renal net glucose release in vivo and its contribution to blood glucose in rats.

Authors:  K Kida; S Nakajo; F Kamiya; Y Toyama; T Nishio; H Nakagawa
Journal:  J Clin Invest       Date:  1978-10       Impact factor: 14.808

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  3 in total

1.  Unsuccessful treatment of severe pyruvate carboxylase deficiency with triheptanoin.

Authors:  C Breen; F J White; C A B Scott; L Heptinstall; J H Walter; S A Jones; A A M Morris
Journal:  Eur J Pediatr       Date:  2013-10-10       Impact factor: 3.183

2.  Pyruvate Carboxylase Deficiency Mimicking Diabetic Ketoacidosis.

Authors:  Pragya Mangla; Poonam Singh Gambhir; Siddhnath Sudhanshu; Priyanka Srivastava; Archana Rai; Vijayalakshmi Bhatia; Shubha R Phadke
Journal:  Indian J Pediatr       Date:  2017-08-23       Impact factor: 1.967

3.  Neonatal pyruvate carboxylase deficiency with renal tubular acidosis and cystinuria.

Authors:  J Oizumi; K N Shaw; T A Giudici; M Carter; G N Donnell; W G Ng
Journal:  J Inherit Metab Dis       Date:  1983       Impact factor: 4.982

  3 in total

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